The Journal of Laryngology and Otology February 1990, Vol. 104, pp. 104-108

Audiological manifestations of Ramsay Hunt syndrome D. M. WAYMAN, M.D.,

H. N. PHAM, M.D.,

F. M. BYL, M.D.,

K. K. ADOUR, M.D.

(Oakland,

California, USA)

Abstract Ramsay Hunt syndrome is known to cause audiological signs and symptoms, including sudden, unexpected hearing loss. We carried out a retrospective review of the audiological manifestations of 186 patients with Ramsay Hunt syndrome, measuring their hearing loss patterns, hyperacusis, tinnitus, herpetic rash, facial paralysis, pain and vertigo. Statistical correlations of these parameters were equated with prognosis. Prognosis for eventual hearing recovery is, in general, excellent. Prognostic indicators of poor hearing recovery include advanced age, retrocochlear hearing loss, male gender, vertigo, and speech frequency hearing loss.

were analyzed with regard to the degree of facial paralysis, initial auditory symptoms, presence of head and neck pain, and presenting clinical signs. Hearing loss patterns, the concomitant signs and symptoms, and ultimate hearing prognosis are discussed.

Introduction The clinical triad of facial palsy, auricular herpes, and hearing loss (Ramsay Hunt syndrome) was first described in 1907 by J. Ramsay Hunt M.D. Hunt postulated that this represented a specific inflammation in the geniculate ganglion, similar to the then recognized Herpes zoster virus (HZV) infection of posterior spinal ganglia. He described auditory symptoms in 19 out of 60 patients, varying from tinnitus aurium and hearing loss, to a more severe Meniere-like syndrome, explained by the anatomical proximity of the HZV-inflamed geniculate ganglion to the acoustic nerve. Today, Ramsay Hunt syndrome is recognized as a polycranial neuritis characterized by damage to sensory and motor nerves, including the auditory-vestibular apparatus. Although clinical findings of the Ramsay Hunt syndrome are well known, indepth analysis of audiological data and their relationship to clinical presentation and prognosis for recovery of hearing has not been undertaken. We performed a retrospective review of 186 Ramsay Hunt syndrome patients. Audiological data

Patients and Methods Between 1966 and 1985, more than 1500 patients were evaluated in the Cranial Nerve Research Clinic at Kaiser Permanente Medical Center, Oakland, California; of these, 186 were diagnosed with Ramsay Hunt syndrome. The diagnosis of Ramsay Hunt syndrome was made based on the classic triad of herpetic rash of the external auditory meatus or pinna, lower motor neuron facial paralysis and auricular pain. Active Herpes zoster infections were confirmed by a four-fold increase between acute and convalescent complement fixation serum titres of varicella-zoster virus. Excluded from study were those patients seen more than 13 days after onset of disease, those with bilateral facial paralysis, 100

60-r I

15-24 25-34 35-44 45-54 55-64 65-74 75-84

80

^H

•mill

85*

Age by Decade

15-24

25-34 35-44 45-54 55-64 65-74 75-84

85*

Age by Decade

FIG. 1 Age distribution by decade of 186 study patients (male, 85 patients; female, 101 patients).

FIG. 2 Age-corrected incidence by decade of Ramsay Hunt syndrome in a health plan group skewed toward a younger population.

Presented in part at the Pacific Coast Oto-Ophthalmologic Society, June 23-28, 1989, Kauai, Hawaii. Accepted for publication: 2 November 1989. 104

105

AUDIOLOGICAL MANIFESTATIONS OF RAMSAY HUNT SYNDROME

TABLE I INITIAL SIGNS AND SYMPTOMS OF RAMSAY HUNT SYNDROME, EXCLUDING AUDIO-VESIBULAR SYMPTOMS, DISTRIBUTED BY TYPE AND SEVERITY OF INITIAL HEARING LOSS IN 1 3 2 PATIENTS

Isolated high-frequency hearing loss Initial signs/symptoms Auricular pain Herpetic rash Cranial polyneuritis (exclude Cr. VII or VIII) Head and neck pain

hearing (n=93)

hearing (n=29)

40(43%) 37 (40%)

21 (72%) 18 (62%)

38 (41%) 37 (40%)

15 (52%) 4(14%)

Speech frequency hearing loss

Moderate (n=5)

Sev/Prof (n=0)

Mild (n=6)

Moderate (n=4)

Sev/Prof (n=3)

p = .01 p = .056

Mild (n=H) 7 (64%) 5 (46%)

2(40%) 4 (80%)

0 0

6 (100%) 4(67%)

3 (75%) 3 (75%)

3 (100%) 2 (67%)

p = .41 p=.O2

4(36%) 1 (9%)

3 (60%) 3 (60%)

0 0

3(50%)

3(75%)

2(67%)

0

0

0

Sev/Prof = severe or profound

those with inadequate follow-up, and those less than five years old. The medical records of each of the 186 subjects were reviewed for data regarding degree of facial paralysis, initial auditory symptoms (hyperacusis, tinnitus, decreased hearing ability), head and neck pain, presenting clinical signs (vertigo, auricular tenderness or erythema, herpetic myocutaneous eruptions, involvement of one or more cranial nerves), and results of audiological studies. Audiograms were performed on 152 (82 per cent) patients, not all of whom had auditory symptoms at the time. Seven patients had a more extensive audiological examination to determine any cochlear or retrocochlear pattern of hearing loss. These studies included shortincrement sensitivity index (SISI), alternate binaural loudness balance (ABLB), Bekesy tracing, tone decay, speech discrimination, and brainstem evoked auditory response. Patients with abnormal audiograms were retested at one- to two-month intervals. In some cases, follow-up testing was obtained several years after initial evaluation. Follow-up was considered adequate only if carried out to a minimum of one month after diagnosis or when complete hearing recovery was recorded. Those subjects lost to late follow-up are included in analysis of early findings but excluded from recovery analysis. Based on initial audiograms, hearing status was categorized using the average decibel levels of the three speech frequencies (500 Hz, 1000 Hz, 2000 Hz): normal hearing, 0-25 dB; mild hearing loss, 26-40 dB; moderate loss, 41-65 dB; severe or profound loss, >66 dB. The low (125 Hz, 250 Hz) and high frequencies (4000 Hz, 8000 Hz) were also recorded. For those patients with bilateral audiological abnormalities, hearing loss was considered cause-related on the side associated with facial paralysis only if there was a loss of 3=10 dB at any frequency compared with the contralateral side. Patients with objective evidence of cause-related hearing loss were treated with oral prednisone. The usual predisone schedule was 60 mg per day in divided

doses for six days and was decreased by 10 mg per day for the next five days. If signs or symptoms of recurrence or progression of disease occurred as the prednisone was tapered (e.g. increasing auricular pain, progression of facial paralysis, or evidence of further facial nerve denervation), the dosage was returned to 60 mg per day for an additional five to seven days. One patient received ACTH followed by oral prednisone, and four patients with mild to moderate facial nerve paralysis received no medical treatment. Results The mean age of patients was 38.7 year, with 102 (55 per cent) female (Fig. 1). A correction for age distribution of the Kaiser Health Plan population demonstrates increasing incidence by decade (Fig. 2). Of 152 initial audiograms, 93 (61 per cent) were normal, 29 (19 per cent) were cause-related abnormal, and 30 (20 per cent) were unrelated abnormal. Audiograms were not performed in the remaining 34 patients. The 30 patients with unrelated abnormal audiograms were excluded from data analysis. Other than audiovestibular symptoms, the most common initial signs and symptoms, in order of frequency, included auricular pain, polycranial nerve involvement, head and neck pain, and herpetic rash (Table I). Auricular pain (p=0.01) and herpetic rash (p=0.056) correlate with the presence of abnormal hearing. Head and neck pain (p=0.018) was inversely correlated with hearing loss. There was no statistically significant relation between the number of individual or total signs or symptoms and severity of speech frequency hearing loss. However, an increased total number of signs or symptoms was associated with increased severity of isolated high-frequency hearing loss. The incidence of auditory symptoms (hyperacusis, tinnitus, and subjective hearing loss) was identical (48 per cent) in the groups with normal (49/93) and causerelated abnormal (13/29) audiograms (Table II). How-

TABLE II INITIAL AUDITORY SYMPTOMS DISTRIBUTED BY TYPE AND SEVERITY OF HEARING LOSS

Normal

Abnormal

Auditory symptoms

(n=93)

(n=29)

Mild

Moderate

Sev/Prof

Mild

Moderate

Sev/Prof

Hyperacusis Hearing loss, tinnitus

44 (47%) 45 (48%)

5 (31%) 5(31%)

3 3

2 2

0 0

6 8

5 5

1 1

Sev/Prof = severe or profound.

Isolated high-frequency hearing loss

Speech frequency hearing loss

106

D. M. WAYMAN, H. N. PHAM, F. M. BYL AND K. K. ADOUR

TABLE III PREVALENCE OF VERTIGO DISTRIBUTED BY TYPE AND SEVERITY OF HEARING LOSS

Symptom

Normal hearing (n=93)

Abnormal hearing (n=29)

Vertigo

5 (5%)

8 (28%)

Isolated high-frequency hearing loss

Speech frequency hearing loss

Mild

Moderate

Sev/Prof

Mild

Moderate

Sev/Prof

2

2

0

1

1

2

ever, those with the cause-related speech frequency hearing loss had a 62 per cent incidence of auditory symptoms, as compared with an incidence of 31 per cent in those with isolated high-frequency loss (p=0.20). Hyperacusis alone was recorded in 46 per cent of the speech-frequency hearing loss group, and in 31 per cent of the isolated high-frequency loss group. Auditory symptoms were more likely to occur with increased severity of isolated high-frequency hearing loss. Vertigo was documented in 8 of 29 (28 per cent) causerelated hearing loss patients, and in only 5 of 93 (5 per cent) normal hearing patients (p=0.002) (Table III). Vertigo was more likely with increased severity of hearing loss in the isolated high-frequency hearing loss group. Of the seven patients who had more extensive audiologic work-up to determine any cochlear or retrochlear pattern of hearing loss, six had audiological findings consistent with cochlear pathology (elevated SISI, ABLB recruitment pattern, type II Bekesy tracing, no evidence of tone decay) (Table IV). One of the six had both cochlear and retrocochlear findings with slightly abnormal tracings and a clinically significant delay in wave V latency. The seventh patient had severe speechfrequency hearing loss with a speech discrimination score of 18 per cent, type IV Bekesy tracing, and positive tone decay, and grossly abnormal brainstem evoked auditory response with marked delay or wave V latency, all suggestive of a retrocochlear hearing loss. No correlation exists between severity of facial paralysis and presence of any hearing loss. The incidence of incomplete paralysis was 68 per cent in the normal hearing group and 62 per cent in the cause-related abnormal hearing group. However, complete facial paralysis occurred in four of five patients with moderate and one of five patients with mild isolated high-frequency hearing loss. Of 16 patients with isolated high-frequency hearing loss, ten were unavailable for follow-up and one had an abnormal cause-unrelated follow-up audiogram. The remaining five patients, all female and less than 64 years of age, had complete recovery of hearing, generally within one month after diagnosis, regardless of severity of initial loss. Of the 13 patients with speech frequency hearing loss, two were unavailable for follow-up and one had inadequate follow-up. Four of the five patients with initial mild speech-frequency loss had complete or near complete hearing recovery; the other had no recovery.

One patient with initial moderate speech-frequency hearing loss had partial hearing recovery. Four with moderate or severe (profound) initial speech-frequency hearing loss had no recovery. Of these, all were male and three were more than 45 years of age. Hearing loss pattern, severity, age, gender, and hearing recovery are shown in Table V. Discussion Ramsay Hunt syndrome, with its triad of herpes auricularis, facial palsy and hearing loss, has been recognized as a clinical entity since 1907. Traditionally this syndrome is attributed to infection by varicella-zoster virus. It is now recognized that Herpes simplex virus may produce lesions that closely resemble Herpes zoster (Weller, 1983). Therefore, Herpes zoster oticus, which has the identical clinical presentation as Ramsay Hunt syndrome, requires virological confirmation of HZV infection for diagnosis. Because confirmation was obtained in fewer than 25 per cent (46 cases) of the patients described here, the diagnosis was audiological manifestations of Ramsay Hunt syndrome instead of Herpes zoster oticus. The herpes virus group is a family of structurally related DNA viruses that includes Herpes simplex type I and type II, varicella-zoster, Epstein-Barr, and cytomegalovirus, and are known to affect hearing (Wilson, 1986). Postulated mechanisms of inner ear pathogenesis include viral labyrinthitis, meningitis, cranial neuropathy, and latent infective ganglionitis (Wilson, 1986). Evidence of specific pathological herpetic involvement of brainstem or temporal bone structures is sketchy. Small series of histopathological temporal bone studies have revealed herpetic inflammatory lesions in variable neural and vascular regions (Denny-Brown et ai, 1944; Blackleyera/., 1967; Zajtchuk etai, 1972). The few histological reports of postaural Herpes zoster temporal bone disease that exist appear to confirm involvement of both sensory and neural structures. Blackley etal. (1967) described variable perineural, intraneural and perivascular lymphocytic or round cell infiltration involving the facial, auditory and vestibular nerves, as well as in the modiolus and organ of Corti in the cochlea. Audiological findings demonstrate both sensory (cochlear) and neural (retrocochlear) hering impairment (Abramovich and Prasher, 1986). In our study, 29 of 122 (24 per cent) had cause-related

TABLE IV SITE LOCALIZATION DISTRIBUTED BY TYPE AND SEVERITY OF HEARING LOSS, IN SEVEN PATIENTS TESTED

Abnormal Site Cochlear Retrocohlear

Speech frequency hearing loss

Isolated high-frequency hearing loss

(n=7)

Mild

Moderate

Sev/Prof

Mild

Moderate

Sev/Prof

6 1

1 0

0 0

0 0

3 0

2 0

0 1

107

AUDIOLOGICAL MANIFESTATIONS OF RAMSAY HUNT SYNDROME

TABLE V HEARING RECOVERY DISTRIBUTED BY TYPE AND SEVERITY OF INITIAL HEARING LOSS IN FIFTEEN PATIENTS WITH COMPLETED FOLLOW-UP

Isolated high-frequency hearing loss (n=5) Hearing recovery Complete

Mild

Moderate

16F, 41F 58F, 61F

61F

Sev/Prof

Speech frequency hearing loss (n=10) Mild

Moderate

Sev/Prof

28F, 47F 64M, 64F

Near complete Partial None

47M

64M 24M, 64M

45M, 67M

Number = age (yr), M = male, F = female.

abnormal hearing. Auditory symptoms of hyperacusis, tinnitus, or subjective hearing loss were present in 59 of 122 (48 per cent) patients analysed. This figure compares with Ramsay Hunt's findings of "acoustic symptoms" in 19 of 60 (32 per cent) cases, and Devriese's (1968) . description of "acoustic symptoms" in 22 of 32 (69 per cent) diagnosed with Herpes zoster oticus. Hyperacusis, the most common auditory symptom, occurred with equal frequency among the normal and abnormal hearing patients. Hyperacusis was previously thought to represent a manifestation of the Vllth cranial nerve dysfunction characterized by loss of the stapedial reflex (McCandless and Schumacher, 1979). This concept is in doubt because hyperacusis is described in patients with an intact stapedial reflex. More current literature attributes the phenomenon of hyperacusis to viral damage of the efferent portion of the VHIth cranial nerve, with consequent loss of inhibitory nerve function (Citron and Adour, 1978). The presence of cochlear and retrocochlear patterns of hearing loss has been reported by others. Harbert and Young (1967) described a patient with poor speech discrimination, positive tone decay and no recruitment by SISI and ABLB who had no hearing recovery, similar to one of our patients. More recently, Abramovich and Prasher (1986) studied 13 Ramsay Hunt syndrome patients with electrophysiological recordings of cochlear and retrocochlear pathology, separately or simultaneously. Vertigo, a known manifestation of Ramsay Hunt syndrome, has been associated with poor prognosis in sudden hearing loss patients (Byl, 1975). Although differing patient selection criteria make comparison of data difficult, this study appears consistent with previous work. Abramovich and Prasher (1986) described vertigo in 11 of 13 (85 per cent) selected Ramsay Hunt syndrome patients, while Devriese (1968) reported "vestibular symptoms" in 23 of 32 (72 per cent). Blackley et al., (1967) have documented histopathological involvement of the modiolus and vestibular nerves. Proctor et al. (1979) described histopathological evidence of viral labyrinthine destruction and vestibular dysfunction in a patient with auricular herpes and hearing loss. Although this study lacks histopathological data, the correlation of vertigo and hearing loss appears consistent with greater extension of viral or lymphocytic inflammatory damage of the audiovestibular system. It seems reasonable to correlate histological and audiological data to conclude that cochlear hearing loss patterns may represent a limited inflammation within the cochlea, while retrocochlear hearing loss and symptoms

of vertigo represent more extensive histopathological disease with a concomitant poorer prognosis. The frequent initial signs and symptoms, auricular pain and herpetic rash, are correlated with initial hearing loss (Table I). However, an insufficient number of study subjects prevents statistical analysis of these signs and symptoms and degree of hearing loss or eventual hearing recovery. It is interesting that the same relative incidence of signs and symptoms was seen in the normal hearing and the cause-related abnormal hearing loss group but with a much greater frequency in the causerelated abnormal group. Hunt described "herpetic otalgia" in his study patients but cited no incidence of actual symptoms. More recent papers also lack such data, except to emphasize the presence of pain when differentiating Ramsay Hunt syndrome from idiopathic (Bell's) palsy (Citron and Adour, 1978; McCandless and Schumacher, 1979).

Conclusion To most patients, the realization of possible persistent hearing loss is disturbing. Thus, when confronted by a patient with the disparate complaints of Ramsay Hunt syndrome, it would be useful for the clinician to separate significant from insignificant complaints in predicting disease outcome. Sensorineural hearing loss was present in 24 per cent of our patients. The overall incidence of residual hearing loss was 5 per cent. Complaints of hyperacusis are distributed equally among the normal and abnormal hearing groups and are of no prognostic value. Auricular pain and herpetic rash are related to initial hearing loss. Factors which appear unfavourable for hearing recovery include older age, retrocochlear hearing loss pattern, presence of vertigo, more severe initial hearing loss, hearing loss in the speech frequency range, and male gender.

References Abramovich, S., Prasher, D.K. (1986) Electrocochleography and brainstem potentials in Ramsay Hunt syndrome. Archives of Otolaryngology Head and Neck Surgery, 112: 925-928. Blackley, B., Friedmann, I., Wright, I. (1967). Herpes zoster auris associated with facial nerve palsy and auditory nerve symptoms. Ada Otolaryngologica, 63: 533-550. Byl, F. (1975) Thirty two cases of sudden profound hearing loss occurring in 1973: Incidence and prognostic findings. Transactions of the American Academy of Ophthalmology and Otolaryngology, 80: 298-305. Citron, D., Adour, K. K. (1978) Acoustic reflex and loudness

108 discomfort in acute facial paralysis. Archives of Otolaryngology, 104: 303-306. Denny-Brown, D., Adams, R. D., Fitzgerald, P. J. (1944) Pathologic features of herpes zoster: A note on "geniculate herpes". Archives of Neurology, 51: 216-231. Devriese, P. P. (1968) Facial paralysis in cephalic herpes zoster. Annals of Otology Rhinology and Laryngology 77: 1101-1119. Harbert, F., Young, I. M. (1967) Audiologic findings in Ramsay Hunt syndrome. Archives of Otolaryngology, 85: 76-83. Hunt, J. R. (1907) On herpetic inflammations of the geniculate ganglion: A new syndrome and its complications. Journal of Nervous and Mental Diseases, 34: 73—96.

McCandless, G. A., Schumacher, M. H. (1979) Auditory dysfunction with facial paralysis. Archives of Otolaryngology, 105: 271-274. Proctor, L., Perlman, H., Lindsay, J., Matz, G. (1979) Acute vestibular paralysis in herpes zoster oticus. Annals of Otology, Rhinology and Laryngology 88: 303-310.

D. M. WAYMAN, H. N. PHAM, F. M. BYL AND K. K. ADOUR

Weller, T. H. (1983) Varicella and herpes zoster: Changing concepts of the natural history, control and importance of a not-sobenign virus. II. New England Journal of Medicine, 309: 1434-1440. Wilson, W. R. (1986) The relationship of the herpes virus family to sudden hearing loss: A prospective clinical study and literature review. Laryngoscope, 96: 870-877. Zajtchuk, J. T., Matz, G. J., Lindsay, J. R. (1972) Temporal bone pathology in herpes oticus. Annals of Otology, Rhinology and Laryngology 81: 331-338. Address for correspondence: Daniel M. Wayman, MD Department of Otolaryngology Kaiser Permanente Medical Center 280 W. MacArthur Blvd. Oakland CA 94611-5693 USA.