Postgraduate Medicine
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Hearing loss the physician's responsibility Michael M. Paparella To cite this article: Michael M. Paparella (1977) Hearing loss the physician's responsibility, Postgraduate Medicine, 62:4, 94-98, DOI: 10.1080/00325481.1977.11714640 To link to this article: http://dx.doi.org/10.1080/00325481.1977.11714640
Published online: 07 Jul 2016.
Submit your article to this journal
View related articles
Citing articles: 1 View citing articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipgm20 Download by: [Australian Catholic University]
Date: 26 July 2017, At: 14:57
•
sympOSIUm
• In the United States, 13 to 14 million people have sufficient hearing Joss to affect their everyday lives. 1 Although figures are not available, it is generally assumed that the majority of these have a significant degree of sensorineural hearing Joss. It is also generally agreed that approximately 10% of the US population, or 22 million people, have a significant hearing or speech problem, or bath. Obviously, a health care problem of this magnitude has profound social and educational implications.
hearing loss the physiclan's responslbllity Michael M. Paparella, MD University of Minnesota Medical School Minneapolis
consider What is the effect on physicians of the new FDA regulation regarding sale of hearing aids? How often should persans with sensorineural hearing loss be seen for follow-up? Why? Under what circumstances may hospitalization for diagnostic workup be necessary in patients with hearing loss?
94
Government Regulation Congress has for years been studying this problem as it affects the many thousands of Americans who need to purchase hearing aids. The result is a new Food and Drug Administration regulation for sale ofhearing aids, effective August 15, 1977, which is of interest to ali physicians. A description of this regulation appears in the February 15, 1977, issue of the Federal Register, in which Sherwin Gardner, acting commissioner of the FDA, states: Hearing loss can resultfrom a number of conditions and diseases for which a hearing aid may not be appropriate therapy. This regulation is designed to assure that ali medical/y treatable conditions which may affect hearing are accurately identified and properly treated before a hearing aid is purchased. One of the provisï"ons of the new regulation is that the purchaser must have obtained medical clearance within the preceding six months, with the option of a waiver for persans over 18 years of age. The six-month limit allows sufficient time for shopping around, yet is short enough to minimize the likelihood of substantial changes in the prospective user's medical condition. The medical statement, which must be from a licensed physician and preferably one specializing in diseases of the ear, simply indicates that the patient has been examined and has been determined to be a candidate for a hearing aid. This article focuses on what is best for the patient from a medical rather th an from a governmental point of view. Nevertheless, the new regulation provides the medical profession with new opportunities and a renewed challenge in management of patients with hearing Joss.
POSTGRADUATE MEDICINE • October 1977 • Vol. 62 • No. 4
Vol. 62 • No. 4 • October 1977 • POSTGRADUATE MEDICINE
95
hearing loss: physician's responsibility - - - - - - - - - - - considerations in differentiai diagnosis of sensorineural hearing loss Congenital deafness
Delayed deafness
Genet le
Genet le
Deafness occurring alone Michel aplasia Mondini aplasia Scheibe aplasia Alexander aplasia
Deafness occurring alone Familial progressive sensorineural deafness Otosclerosis "Presbycusis"
Deafness occurring with other abnormalities Waardenburg syndrome (deafness may be delayed) Albinism Hyperpigmentation Onychodystrophy Pendred syndrome Jervell and Lange-Nielsen syndrome Usher syndrome Chromosomal abnormalities Trisomy 13-15 syndrome Trisomy 18 syndrome
Nongenetlc Deafness occurring alone Ototoxic poisoning (eg, streptomycin, quinine) Deafness occurring with other abnormalities Viral infection (eg, maternai rubella) Bacterial infection Ototoxic poisoning (eg, thalidomide) Metabolic disorders (eg, cretinism) Erythroblastosis fetalis Radiation (first trimester) Prematurity Birth trauma, anoxia
Signs and Symptoms Hearing loss is a symptom (or a finding if documented on an audiogram) of otologie disease. It can be of the conductive, sensorineural, or mixed type. That most conductive !osses are correctable medically or surgically is generally understood by the medical profession. lt is not generally understood, however, that sensorineural hearing loss also may benefit from medical diagno'sis and management, as researchers are discovering is true in more and more cases. For this reason, the following discussion emphasizes sensorineural hearing loss, although the principles apply to all forms. The primary symptoms of inn er ear disease are hearing loss, tinnitus, and vertigo. Hearing loss and tinnitus arise from the front part of the inn er ear (auditory labyrinth), which is related to hearing, while vertigo arises from the posterior portion of the inn er ear (vestibu-
96
Deafness occurring with other abnormalities Alport syndrome Hurler syndrome (eg, gargoylism) Klippei-Feil syndrome Refsum syndrome Alstrom syndrome Paget disease Richards-Rundel syndrome Von Recklinghausen disease Crouzon disease
Nongenetlc lnflammatory disease Bacterial (eg, labyrinthitis and otitis media) Viral (eg, measles, mumps, influenza, labyrinthitis) Spirochetal (eg, congenital and acquired syphilis) Noninflammatory disease Ototoxic poisoning Neoplastic disorders (eg, leukemia, lymphoma, peripheral and central ear tu mors) Traumatic injury (eg, acoustic trauma, temporal bone fractures) Metabolic disorders (eg, hypothyroidism, allergies, Maniere disease) Vascular insufficiency (eg, sudden deafness) CNS disease (eg, multiple sclerosis) "Presbycusis"
lar labyrinth), which relates to maintenance of balance and equilibrium.
Responsibility for Diagnosis and Treatment Every person with a hearing loss requires first and foremost a medical diagnosis and, insofar as is possible, medical management. Physicians in general and the otolaryngologist or otologist in particular are the only professionals capable of performing these functions. To assume their responsibility in this area, sorne physicians may find they need to expand their knowledge of hearing. The other key profession al qualified to deal with hearing loss is the medical audiologist, who provides information regarding function which is useful in diagnosis and is particularly helpful in rehabilitation. In every case of hearing loss there is an underlying problem or disease which must be identified and, if possible, treated medically
POSTQRADUATE MEDICINE • October 1977 • Vol. 62 • No. 4
Michael M. Paparella
or surgically. Too many paramedical persons conceive of management of hearing loss as dealing with the symptoms or findings perse, and thus the underlying disease is circumvented.
Dr Paparella is chairman, department of otolaryngology, University of Minnesota Medical School, MinneapOlis.
Differentiai Diagnosis Earl y recognition of hearing loss is the responsibility of the patient, his or ber family and teachers, and health care professionalsiri short, all of us. When a hearing loss is suspected, the patient should be seen by a physician who is trained in and interested in ear problems, preferably one who is working with an audiologist. A careful medical history and physical examination of the head, the neck, and especially the ear are necessary for every patient with suspected hearing loss or deafness. Ancillary studies are selected as needed, but audiology is an essential aid to diagnosis. The vast majority of hearing loss problems are diagnosed and managed effectively in an outpatient setting rather th an in the hospital and at considerably less cost. The primary emphasis of the diagnostic workup is to rule out ali possible extrinsic causes of deafness or hearing loss, after which the diagnosis, by exclusion, is often intrinsic or genetic deafness. Among the literally hundreds of extrinsic causes are infection, drugs (ototoxicity), tumors, trauma, neurologie problems, congenital conditions, and metabolic abnormalities. Whether hearing loss is conductive, sensorineural, or mixed is determined through otologie and audiologie assessment. Conductive tosses, in the main, can be treated and corrected medically or surgie ally. Diagnostic microotoscopy and therapeutic microsurgery are of immense benefit in this type of hearing loss. As more is leamed about sensorineural hearing loss, more problems are discovered which can be managed medically. Routine diagnostic workup-I will now briefly describe my approach to medical diagnosis for patients with a sensorineural hearing loss, using traditional methods. Expensive tests, such as polytomography and electronystagmography, are usually not required. By far the most important part of the diagnostic process is his tory taking. Second in importance is the physical examination, together with audiometry and other audiologie
procedures. Results of these alone lead to a definitive or a working diagnosis for the vast majority of sensorineural hearing problems. In initiating a patient workup, the first question I try to answer is, "Did the hearing loss occur at time of birth (congenital) or subsequently (delayed)?" Congenital deafness implies aplasia or dysgenesis of the cochlear duct and for this reason should not be progressive in most cases. Delayed, or acquired, hearing loss relates to degenerative changes of the sense organs and may be progressive. There is no reason why a progressive or delayed hearing loss cannot superimpose itself on a congenital problem. The second question I try to answer is, "Is the hearing loss genetic (hereditary) or nongenetic?'' This is important because profound hearing loss in children is genetic in approximately half of all cases. The third question relates to other anomalies occurring with the deafness. These syndromes can be seen at birth (congenital or genetic deafness), or may be of delayed onset. When 1 have identified the sensorineural hearing loss as congenital or delayed and genetic or nongenetic, I can th en zero in on the specifie diagnosis. By careful questioning I can usually identify the specifie disease responsible for the hearing loss. A more detailed differentiai diagnosis of sensorineural hearing loss possible with this approach is shown in the table. After a working or differentiai diagnosis is achieved, the hearing loss must be identified as stable, progressive, or fluctuant. It is possible for a progressive sensorineural hearing loss to become stable or for a fluctuant loss to become progressive. In addition to the dynamic changes which might occur, new conditions with additive effects may appear after the initial diagnosis. For these reasons, it is important that all patients with sensorineural hearing loss be seen periodically, preferably yearly but at least every two years. Additional studies-Rarely, a patient with profound sensorineural hearing loss or deafness requires further studies which may be best accomplished in the hospital. The pro-
Vol. 82 • No. 4 • October 1977 • POSTORADUATE MEDICINE
97
hearing loss: physician's responslbillty - - tocol used for diagnostic workup of the hospitalized patient is included in the article by Dr Meyerhoff. * Indications for hospitalization and further study may include absence of a working diagnosis for certain cases of idiopathie deafness; systemic problems which may cause deafness (eg, deafness associated with renal disease, thyroid disease, syphilis, metabolic disease); fluctuant hearing loss suggestive of endolymphatic hydrops; preliminary findings suggestive of a retrocochlear or CNS problem; or hearing loss of sudden onset, especially if the patient is seen shortly after its occurrence.
Summary Hearing loss is a very important and cornmon problem in the United States, affecting 13 to 14 million people. It is primarily a medical problem, requiring diagnosis and, when indicated, treatment by a physician, preferably in collaboration with an audiologist. History taking is the most important part of the diagnostic process, followed by a physical examination with special attention to the head, neck, and ears. The primary object of the workup is to exclude all possible extrinsic causes of hearing loss. Intrinsic or genetic deafness is a diagnosis of exclusion. After the hearing loss bas been classified as congenital or delayed and as genetic or nongenetic, a differentiai or working diagnosis can be made in all cases and a specifie diagnosis in most cases. The hearing loss must then be identified as stable, progressive, or fluctuant over time. Follow-up at least every two years is important to check for possible changes in status. • Address reprint requests to Michael M. Paparella, MD, Department of Otolaryngology, University of Minnesota Medical School, A-605 Mayo Memorial Bldg, Minneapolis, MN 55455. ReadySource on hearing loss appears on page 143. CME Credit Quiz on hearing loss begins on page 147. *See page 109.
Reference 1. National census of the deaf population; interview responses. In Schein ID, Delk MT: The DeafPopulation of the United States. Silver Spring, Md, National Association of the Deaf, 1974
98
PRONEsm• TABLETS Procalnamlde Hydroc:bloride Tablets PRONESTYL•CAPSULES Procalnamlde Hydroc:bloride Capsules U.S.P. The prolonged administration of procainamide often leads to the development of a positive anti-nuclear antibody (ANA) test with or without symptoms of lupus erythematosus-Iike syndrome. If a positive ANA titer develops, the benefit/risk ratio related to continued procainamide therapy should be assessed. This may necessita te considerations of alternative anti-arrhythmic therapy. DESCRIPTION: .Pronestyl (Procainamide Hydrochloride) is the amide analogue of procaine hydrochloride and is available for oral administration as capsules and veneer-coated tablets providing 250 mg., 375 mg., and 500 mg. procainamide hydrochloride. CONTRAINDICATIONS: In patients with myasthenia gravis and where a hypersensitivity to procainamide exists; bear in mind cross sensitivity to procaine and related drugs. Should not be given to patients with complete atrioventricular heart block. Contraindicated in cases of second degree and third degree A·V block unless an electrical pacemaker is operative. ADMINISTRATION: Should procainamide therapy be continued for any appreciable period, electrocardiograms should be made occasionally to determine its further need. PRECAUTIONS: Evidence of untoward myocardial response should be carefully watched for in ali patients. In the presence of myocardial damage with atrial fibrillation or flutter, the ventricular rate may increase suddenly as the atrial rate is slowed; adequate digitalization reduces but does not abolish this danger. Ventricular tachysystole is particularly hazardous if myocardial damage exists. The dislodgment of mural thrombi producing an embolie episode may occur in correcting atrial fibrillation due to the forceful contractions of the atrium. Extreme caution is required in attempting to adjust the heart rate when ventricular tachycardia bas occurred during an occlusive coronary episode or where the use of procainamide may result in additional depression of conduction and ventricular asystole or fibrillation as in second degree and third degree A-V block, bundle branch block, or severe digitalis intoxication. Bear in mind when treating ventricular arrhythmias in patients with severe organic heart disease and ventricular tachycardia that complete heart block, which may be difficult to diagnose, may be present. Since asystole may result if the ventricular rate is significantly slowed without attainment of regular atrioventricular conduction, procainamide should be stopped and the patient reevaluated. In the presence of both Ii ver and kidney damage, normal dosage may produce symptoms of overdosage-principally ventricular tachycardia and severe hypotension. A syndrome resembling lupus erythematosus bas been reported with maintenance procainamide therapy. Common symptoms are polyarthralgia, arthritis and pleuritic pain. Pever, myalgia, skin lesions, pleural effusion and pericarditis may also occur. Rare cases of thrombocytopenia or Coombs-positive hemolytic anemia, possibly related to this syndrome, have been reported. Measure anti· nuclear antibody titers at regular intervals in patients -on procainamide for extended periods of time or in whom symptoms suggestive of Iupus-Iike reaction appear; in event of rising titer (atlti-nuclear antibody) or clinical symptoms of LE, assess the benefit/risk ratio related to continued procainamide therapy (see boxed Waming). Steroid therapy may be effective if discontinuation of procainamide does not cause remission of symptoms. If the syndrome develops in a patient with recurrent life-threatening arrhythmias not otherwise controllable, steroid-suppressive therapy may be used concomitantly with procainamide. ADVERSE REACTIONS: Hypotension is rare with oral administration. Serious disturbances of cardiac rhythm such as ventricular asystole or fibrillation are more common with !.V. administration. Large oral doses may sometimes produce anorexia, nausea, urticaria, and/or prurit us. A syndrome resembling lupus erythematosus bas been reported (see Precautions). Reactions consisting of fever and chills have been reported, including a case with nausea, vomiting, abdominal pain, acute hepatomegaly, and arise in serum glutamic oxaloacetic transaminase following single doses of the drug. Agranulocytosis bas been occasionally reported following repeated use of the drug, and deaths have occurred. Therefore, routine blood counts are advisable during maintenance procainamide therapy; and the patient should be instructed to report any soreness of the mouth, throat, or gums, unexplained fever or any symptoms of upper respiratory tract infection. If any of these symptoms should occur and leukocyte counts indicate cellular depression, procainamide therapy should be discontinued and appropriate treatment should be instituted immediately. Bitter taste, diarrhea, weakness, mental depression, giddiness, psychosis with hallucinations, and hypersensitivity reactions such as angioneurotic edema and maculopapular rash have been reported. The package insert should be read carefully to become familiar with the recommended dosage for the indicated conditions and for full prescribing information. Priceless Ingredient of every product SQU1884!> 'The is the honor and integrity of its maker.'"'
Cl
tm E. R. Squibb & Sons. Inc. 447·504
POSTGRADUATE MEDICINE • October 19n • Vol. 62 • No. 4
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Hearing loss the physician's responsibility Michael M. Paparella To cite this article: Michael M. Paparella (1977) Hearing loss the physician's responsibility, Postgraduate Medicine, 62:4, 94-98, DOI: 10.1080/00325481.1977.11714640 To link to this article: http://dx.doi.org/10.1080/00325481.1977.11714640
Published online: 07 Jul 2016.
Submit your article to this journal
View related articles
Citing articles: 1 View citing articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipgm20 Download by: [Australian Catholic University]
Date: 26 July 2017, At: 14:57
•
sympOSIUm
• In the United States, 13 to 14 million people have sufficient hearing Joss to affect their everyday lives. 1 Although figures are not available, it is generally assumed that the majority of these have a significant degree of sensorineural hearing Joss. It is also generally agreed that approximately 10% of the US population, or 22 million people, have a significant hearing or speech problem, or bath. Obviously, a health care problem of this magnitude has profound social and educational implications.
hearing loss the physiclan's responslbllity Michael M. Paparella, MD University of Minnesota Medical School Minneapolis
consider What is the effect on physicians of the new FDA regulation regarding sale of hearing aids? How often should persans with sensorineural hearing loss be seen for follow-up? Why? Under what circumstances may hospitalization for diagnostic workup be necessary in patients with hearing loss?
94
Government Regulation Congress has for years been studying this problem as it affects the many thousands of Americans who need to purchase hearing aids. The result is a new Food and Drug Administration regulation for sale ofhearing aids, effective August 15, 1977, which is of interest to ali physicians. A description of this regulation appears in the February 15, 1977, issue of the Federal Register, in which Sherwin Gardner, acting commissioner of the FDA, states: Hearing loss can resultfrom a number of conditions and diseases for which a hearing aid may not be appropriate therapy. This regulation is designed to assure that ali medical/y treatable conditions which may affect hearing are accurately identified and properly treated before a hearing aid is purchased. One of the provisï"ons of the new regulation is that the purchaser must have obtained medical clearance within the preceding six months, with the option of a waiver for persans over 18 years of age. The six-month limit allows sufficient time for shopping around, yet is short enough to minimize the likelihood of substantial changes in the prospective user's medical condition. The medical statement, which must be from a licensed physician and preferably one specializing in diseases of the ear, simply indicates that the patient has been examined and has been determined to be a candidate for a hearing aid. This article focuses on what is best for the patient from a medical rather th an from a governmental point of view. Nevertheless, the new regulation provides the medical profession with new opportunities and a renewed challenge in management of patients with hearing Joss.
POSTGRADUATE MEDICINE • October 1977 • Vol. 62 • No. 4
Vol. 62 • No. 4 • October 1977 • POSTGRADUATE MEDICINE
95
hearing loss: physician's responsibility - - - - - - - - - - - considerations in differentiai diagnosis of sensorineural hearing loss Congenital deafness
Delayed deafness
Genet le
Genet le
Deafness occurring alone Michel aplasia Mondini aplasia Scheibe aplasia Alexander aplasia
Deafness occurring alone Familial progressive sensorineural deafness Otosclerosis "Presbycusis"
Deafness occurring with other abnormalities Waardenburg syndrome (deafness may be delayed) Albinism Hyperpigmentation Onychodystrophy Pendred syndrome Jervell and Lange-Nielsen syndrome Usher syndrome Chromosomal abnormalities Trisomy 13-15 syndrome Trisomy 18 syndrome
Nongenetlc Deafness occurring alone Ototoxic poisoning (eg, streptomycin, quinine) Deafness occurring with other abnormalities Viral infection (eg, maternai rubella) Bacterial infection Ototoxic poisoning (eg, thalidomide) Metabolic disorders (eg, cretinism) Erythroblastosis fetalis Radiation (first trimester) Prematurity Birth trauma, anoxia
Signs and Symptoms Hearing loss is a symptom (or a finding if documented on an audiogram) of otologie disease. It can be of the conductive, sensorineural, or mixed type. That most conductive !osses are correctable medically or surgically is generally understood by the medical profession. lt is not generally understood, however, that sensorineural hearing loss also may benefit from medical diagno'sis and management, as researchers are discovering is true in more and more cases. For this reason, the following discussion emphasizes sensorineural hearing loss, although the principles apply to all forms. The primary symptoms of inn er ear disease are hearing loss, tinnitus, and vertigo. Hearing loss and tinnitus arise from the front part of the inn er ear (auditory labyrinth), which is related to hearing, while vertigo arises from the posterior portion of the inn er ear (vestibu-
96
Deafness occurring with other abnormalities Alport syndrome Hurler syndrome (eg, gargoylism) Klippei-Feil syndrome Refsum syndrome Alstrom syndrome Paget disease Richards-Rundel syndrome Von Recklinghausen disease Crouzon disease
Nongenetlc lnflammatory disease Bacterial (eg, labyrinthitis and otitis media) Viral (eg, measles, mumps, influenza, labyrinthitis) Spirochetal (eg, congenital and acquired syphilis) Noninflammatory disease Ototoxic poisoning Neoplastic disorders (eg, leukemia, lymphoma, peripheral and central ear tu mors) Traumatic injury (eg, acoustic trauma, temporal bone fractures) Metabolic disorders (eg, hypothyroidism, allergies, Maniere disease) Vascular insufficiency (eg, sudden deafness) CNS disease (eg, multiple sclerosis) "Presbycusis"
lar labyrinth), which relates to maintenance of balance and equilibrium.
Responsibility for Diagnosis and Treatment Every person with a hearing loss requires first and foremost a medical diagnosis and, insofar as is possible, medical management. Physicians in general and the otolaryngologist or otologist in particular are the only professionals capable of performing these functions. To assume their responsibility in this area, sorne physicians may find they need to expand their knowledge of hearing. The other key profession al qualified to deal with hearing loss is the medical audiologist, who provides information regarding function which is useful in diagnosis and is particularly helpful in rehabilitation. In every case of hearing loss there is an underlying problem or disease which must be identified and, if possible, treated medically
POSTQRADUATE MEDICINE • October 1977 • Vol. 62 • No. 4
Michael M. Paparella
or surgically. Too many paramedical persons conceive of management of hearing loss as dealing with the symptoms or findings perse, and thus the underlying disease is circumvented.
Dr Paparella is chairman, department of otolaryngology, University of Minnesota Medical School, MinneapOlis.
Differentiai Diagnosis Earl y recognition of hearing loss is the responsibility of the patient, his or ber family and teachers, and health care professionalsiri short, all of us. When a hearing loss is suspected, the patient should be seen by a physician who is trained in and interested in ear problems, preferably one who is working with an audiologist. A careful medical history and physical examination of the head, the neck, and especially the ear are necessary for every patient with suspected hearing loss or deafness. Ancillary studies are selected as needed, but audiology is an essential aid to diagnosis. The vast majority of hearing loss problems are diagnosed and managed effectively in an outpatient setting rather th an in the hospital and at considerably less cost. The primary emphasis of the diagnostic workup is to rule out ali possible extrinsic causes of deafness or hearing loss, after which the diagnosis, by exclusion, is often intrinsic or genetic deafness. Among the literally hundreds of extrinsic causes are infection, drugs (ototoxicity), tumors, trauma, neurologie problems, congenital conditions, and metabolic abnormalities. Whether hearing loss is conductive, sensorineural, or mixed is determined through otologie and audiologie assessment. Conductive tosses, in the main, can be treated and corrected medically or surgie ally. Diagnostic microotoscopy and therapeutic microsurgery are of immense benefit in this type of hearing loss. As more is leamed about sensorineural hearing loss, more problems are discovered which can be managed medically. Routine diagnostic workup-I will now briefly describe my approach to medical diagnosis for patients with a sensorineural hearing loss, using traditional methods. Expensive tests, such as polytomography and electronystagmography, are usually not required. By far the most important part of the diagnostic process is his tory taking. Second in importance is the physical examination, together with audiometry and other audiologie
procedures. Results of these alone lead to a definitive or a working diagnosis for the vast majority of sensorineural hearing problems. In initiating a patient workup, the first question I try to answer is, "Did the hearing loss occur at time of birth (congenital) or subsequently (delayed)?" Congenital deafness implies aplasia or dysgenesis of the cochlear duct and for this reason should not be progressive in most cases. Delayed, or acquired, hearing loss relates to degenerative changes of the sense organs and may be progressive. There is no reason why a progressive or delayed hearing loss cannot superimpose itself on a congenital problem. The second question I try to answer is, "Is the hearing loss genetic (hereditary) or nongenetic?'' This is important because profound hearing loss in children is genetic in approximately half of all cases. The third question relates to other anomalies occurring with the deafness. These syndromes can be seen at birth (congenital or genetic deafness), or may be of delayed onset. When 1 have identified the sensorineural hearing loss as congenital or delayed and genetic or nongenetic, I can th en zero in on the specifie diagnosis. By careful questioning I can usually identify the specifie disease responsible for the hearing loss. A more detailed differentiai diagnosis of sensorineural hearing loss possible with this approach is shown in the table. After a working or differentiai diagnosis is achieved, the hearing loss must be identified as stable, progressive, or fluctuant. It is possible for a progressive sensorineural hearing loss to become stable or for a fluctuant loss to become progressive. In addition to the dynamic changes which might occur, new conditions with additive effects may appear after the initial diagnosis. For these reasons, it is important that all patients with sensorineural hearing loss be seen periodically, preferably yearly but at least every two years. Additional studies-Rarely, a patient with profound sensorineural hearing loss or deafness requires further studies which may be best accomplished in the hospital. The pro-
Vol. 82 • No. 4 • October 1977 • POSTORADUATE MEDICINE
97
hearing loss: physician's responslbillty - - tocol used for diagnostic workup of the hospitalized patient is included in the article by Dr Meyerhoff. * Indications for hospitalization and further study may include absence of a working diagnosis for certain cases of idiopathie deafness; systemic problems which may cause deafness (eg, deafness associated with renal disease, thyroid disease, syphilis, metabolic disease); fluctuant hearing loss suggestive of endolymphatic hydrops; preliminary findings suggestive of a retrocochlear or CNS problem; or hearing loss of sudden onset, especially if the patient is seen shortly after its occurrence.
Summary Hearing loss is a very important and cornmon problem in the United States, affecting 13 to 14 million people. It is primarily a medical problem, requiring diagnosis and, when indicated, treatment by a physician, preferably in collaboration with an audiologist. History taking is the most important part of the diagnostic process, followed by a physical examination with special attention to the head, neck, and ears. The primary object of the workup is to exclude all possible extrinsic causes of hearing loss. Intrinsic or genetic deafness is a diagnosis of exclusion. After the hearing loss bas been classified as congenital or delayed and as genetic or nongenetic, a differentiai or working diagnosis can be made in all cases and a specifie diagnosis in most cases. The hearing loss must then be identified as stable, progressive, or fluctuant over time. Follow-up at least every two years is important to check for possible changes in status. • Address reprint requests to Michael M. Paparella, MD, Department of Otolaryngology, University of Minnesota Medical School, A-605 Mayo Memorial Bldg, Minneapolis, MN 55455. ReadySource on hearing loss appears on page 143. CME Credit Quiz on hearing loss begins on page 147. *See page 109.
Reference 1. National census of the deaf population; interview responses. In Schein ID, Delk MT: The DeafPopulation of the United States. Silver Spring, Md, National Association of the Deaf, 1974
98
PRONEsm• TABLETS Procalnamlde Hydroc:bloride Tablets PRONESTYL•CAPSULES Procalnamlde Hydroc:bloride Capsules U.S.P. The prolonged administration of procainamide often leads to the development of a positive anti-nuclear antibody (ANA) test with or without symptoms of lupus erythematosus-Iike syndrome. If a positive ANA titer develops, the benefit/risk ratio related to continued procainamide therapy should be assessed. This may necessita te considerations of alternative anti-arrhythmic therapy. DESCRIPTION: .Pronestyl (Procainamide Hydrochloride) is the amide analogue of procaine hydrochloride and is available for oral administration as capsules and veneer-coated tablets providing 250 mg., 375 mg., and 500 mg. procainamide hydrochloride. CONTRAINDICATIONS: In patients with myasthenia gravis and where a hypersensitivity to procainamide exists; bear in mind cross sensitivity to procaine and related drugs. Should not be given to patients with complete atrioventricular heart block. Contraindicated in cases of second degree and third degree A·V block unless an electrical pacemaker is operative. ADMINISTRATION: Should procainamide therapy be continued for any appreciable period, electrocardiograms should be made occasionally to determine its further need. PRECAUTIONS: Evidence of untoward myocardial response should be carefully watched for in ali patients. In the presence of myocardial damage with atrial fibrillation or flutter, the ventricular rate may increase suddenly as the atrial rate is slowed; adequate digitalization reduces but does not abolish this danger. Ventricular tachysystole is particularly hazardous if myocardial damage exists. The dislodgment of mural thrombi producing an embolie episode may occur in correcting atrial fibrillation due to the forceful contractions of the atrium. Extreme caution is required in attempting to adjust the heart rate when ventricular tachycardia bas occurred during an occlusive coronary episode or where the use of procainamide may result in additional depression of conduction and ventricular asystole or fibrillation as in second degree and third degree A-V block, bundle branch block, or severe digitalis intoxication. Bear in mind when treating ventricular arrhythmias in patients with severe organic heart disease and ventricular tachycardia that complete heart block, which may be difficult to diagnose, may be present. Since asystole may result if the ventricular rate is significantly slowed without attainment of regular atrioventricular conduction, procainamide should be stopped and the patient reevaluated. In the presence of both Ii ver and kidney damage, normal dosage may produce symptoms of overdosage-principally ventricular tachycardia and severe hypotension. A syndrome resembling lupus erythematosus bas been reported with maintenance procainamide therapy. Common symptoms are polyarthralgia, arthritis and pleuritic pain. Pever, myalgia, skin lesions, pleural effusion and pericarditis may also occur. Rare cases of thrombocytopenia or Coombs-positive hemolytic anemia, possibly related to this syndrome, have been reported. Measure anti· nuclear antibody titers at regular intervals in patients -on procainamide for extended periods of time or in whom symptoms suggestive of Iupus-Iike reaction appear; in event of rising titer (atlti-nuclear antibody) or clinical symptoms of LE, assess the benefit/risk ratio related to continued procainamide therapy (see boxed Waming). Steroid therapy may be effective if discontinuation of procainamide does not cause remission of symptoms. If the syndrome develops in a patient with recurrent life-threatening arrhythmias not otherwise controllable, steroid-suppressive therapy may be used concomitantly with procainamide. ADVERSE REACTIONS: Hypotension is rare with oral administration. Serious disturbances of cardiac rhythm such as ventricular asystole or fibrillation are more common with !.V. administration. Large oral doses may sometimes produce anorexia, nausea, urticaria, and/or prurit us. A syndrome resembling lupus erythematosus bas been reported (see Precautions). Reactions consisting of fever and chills have been reported, including a case with nausea, vomiting, abdominal pain, acute hepatomegaly, and arise in serum glutamic oxaloacetic transaminase following single doses of the drug. Agranulocytosis bas been occasionally reported following repeated use of the drug, and deaths have occurred. Therefore, routine blood counts are advisable during maintenance procainamide therapy; and the patient should be instructed to report any soreness of the mouth, throat, or gums, unexplained fever or any symptoms of upper respiratory tract infection. If any of these symptoms should occur and leukocyte counts indicate cellular depression, procainamide therapy should be discontinued and appropriate treatment should be instituted immediately. Bitter taste, diarrhea, weakness, mental depression, giddiness, psychosis with hallucinations, and hypersensitivity reactions such as angioneurotic edema and maculopapular rash have been reported. The package insert should be read carefully to become familiar with the recommended dosage for the indicated conditions and for full prescribing information. Priceless Ingredient of every product SQU1884!> 'The is the honor and integrity of its maker.'"'
Cl
tm E. R. Squibb & Sons. Inc. 447·504
POSTGRADUATE MEDICINE • October 19n • Vol. 62 • No. 4
