Recurrent

Meningitis and Mondini Dysplasia

Laurie A. Ohlms, MD; Morven S. Edwards, MD; Edward O. Mason, MD; Makoto Igarashi, MD; Bobby R. Alford, MD; Richard J. H. Smith, MD

\s=b\ Three infants with cerebrospinal fluid otorrhea and recurrent meningitis due to Mondini dysplasia have been treated at Baylor College of Medicine, Houston, Tex; 39 other patients with this association have been described. This review highlights the characteristics and embryologic basis of the malformation. The clinical evaluation of an infant with recurrent meningitis is outlined, and the importance of brain-stem response audiometry and thincut computed tomography of the temporal bones is discussed. Surgical management depends on the functional status of the dysplastic ear; usually, a stapedectomy is performed and the vestibule is obliterated. Although the surgical failure rate is 30% after one procedure, early identification of the dysplasia and prompt surgical management diminish morbidity and mortality and permit early habilitation of the child with congenital hearing impairment. (Arch Otolaryngol Head Neck Surg.

1990;116:608-612)

Four major patterns

of congenital malformation are com¬ monly recognized. These patterns repinner

ear

Accepted for publication November 13,1989. From the Departments of Otorhinolaryngology and Communicative Sciences (Drs Ohlms, Igarashi, Alford, and Smith) and Pediatrics, Divison of Infectious Diseases (Drs Edwards and Mason), Baylor College of Medicine, Houston, Tex. Presented in part at the annual meeting of the American Society of Pediatric Otolaryngology, San Diego, Calif, May 17-20, 1989. Reprint requests to Department of Otolaryngology and Communicative Sciences, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030 (Dr Smith)

points along a continuum, mak¬ ing specific "classification" of a given anomaly often difficult. The most se¬ vere aplasia, the Michel, is character¬ ized by complete failure of inner ear development. Incomplete formation of the bony and membranous labyrinths is known as Mondini dysplasia. Scheibe dysplasia, the most commonly encountered, is characterized by ab¬ normal development of only the mem¬ branous pars inferior. Alexander dys¬ plasia is the least severe type of mal¬ formation and consists of aplasia of the cochlear duct; usually only the resent

basal turn is affected. Typically, these malformations are associated with some degree of hear¬ ing impairment. In addition, the Mon¬ dini malformation can be associated with cerebrospinal fluid (CSP) otor¬ rhea and recurrent meningitis. Three patients with this defect have been seen at Baylor College of Medicine, Houston, Tex. Two have been previ¬ ously described and are briefly dis¬ cussed here; the third is described in detail.1 2 The morphological features, clinical presentation, and treatment options are described. REPORT OF CASES

Case 1.—An 18-month-old boy was seen after his second episode of pneumococcal meningitis. Hearing was absent in the right ear with only minimal low-frequency re¬ sponses present in the left. Physical exam¬ ination revealed a pale, bulging right tym-

panic membrane; following a right myringotomy, a profuse flow of clear fluid was noted. Middle ear exploration confirmed CSF leaking from the oval window. The in¬ cus and stapes were removed, and the en¬ larged vestibule was packed with temporalis fascia. A small piece of cortical bone was used to "cork" the oval window, and the middle ear was packed with Surgicel. Re¬ covery was uneventful. At the age of 9 years, the patient suffered a third attack of meningitis; evaluation at that time failed to

reveal a CSF leak. Case 2.—A 1-year-old boy presented with pneumococcal meningitis and bilateral acute suppurative otitis media. After bilat¬ eral myringotomies, pus, followed by a co¬ pious flow of CSF, was noted in the right ear. Auditory testing showed no response in that ear, although in the left a response was obtained at 60-dB hearing loss. Computed tomography confirmed bilateral Mondini malformations. At surgery, CSF was leak¬ ing from a perforation in the anterior stapes footplate and round-window mem¬ brane. The incus and stapes were removed, and the large dilated vestibule was packed with temporalis fascia; the oval and round windows were blocked with conchal carti¬ lage. Recovery was uneventful. Case 3.—A 15-month-old boy was seen at Texas Children's Hospital, Houston, after his second episode of meningitis. By his¬ tory, he was a term infant who weighed 3.4 kg and was delivered by cesarean section. Meconium staining and aspiration occurred at birth, and he was placed on a ventilator for the first 2 days of life. By day 4, he was discharged from the hospital. He did well until 1 year of age, when he developed pneumococcal meningitis after an episode

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Fig 1.—Thin-cut computed tomographic scan showing characteristic appearance on Mondini malformation (left).

Fig 2.— High-power view of the stapes show¬ ing mucosal sac arising from footplate (case 3).

Fig 3.—Defects in stapes footplate (case 3).

of otitis media. This episode resolved with ampicillin sodium therapy. Over the next 2 months, a continuous regimen of oral anti¬ biotics was used to treat persistent otitis media with effusion. In late January 1988, the patient developed another episode of acute otitis media, again followed by pneu¬ mococcal meningitis. He was transferred to Texas Children's Hospital for further eval¬ uation. Physical examination confirmed the presence of a left serous otitis. The white blood cell count was elevated to 19 X 10VL, and cultures of blood and CSF yielded Streptococcus pneumoniae. Thin-cut com¬ puted tomographic scanning revealed a Mondini malformation of the left ear, and auditory testing showed no response to air or bone conduction on that side (Fig 1). Exploration of the left middle ear re¬ vealed a fluid-filled mucosal sac obscuring the stapedial footplate; clear fluid was leak¬ ing from the sac. Following stapedectomy, the enlarged vestibule was obliterated with temporalis muscle and fascia; the incus, used to cork the oval window, was anchored in position by the malleus. Inspection of the stapes showed an oval-shaped bony defect in the footplate (Figs 2 and 3). Postoperatively, the patient did well and has had no further episodes of meningitis.

year-old boy, born deaf, who developed

dini malformation to the Royal Society of Medicine. His clinical description suggests that the child had the Wildervanck form of the Klippel-Feil syn¬ drome. These temporal bone sections now reside in the collection at the Uni¬ versity of Zurich (Switzerland). Several temporal bone studies have confirmed the pathologic changes de¬

COMMENT

mann5 reported another case of incom¬ plete cochlear development associated with congenital deafness. He named the defect after Mondini. Twentythree years later, Fraser6 presented a report of a child with bilateral Mon-

In 1791, Carlo Mondini3 presented a paper before the Academy of Sciences of the University of Bologna (Italy) entitled, "An Anatomical Dissection of a Boy Born Deaf." He described an 8-

gangrene after he foot by a wagon; in

the a few days, he died. Mondini immediately applied himself "to the examination of the organs of hearing, and was careful to the ut¬ most of [his] ability that not even the smallest part should escape [his] dili¬ gent scrutiny." He noted that the ex¬ ternal and middle ears appeared nor¬ mal but, "As regards to the vestibule, there was nothing in its shape which indicated any defect, except that it had acquired a greater than normal size." The cochlea "was exceedingly flawed. According to its nature it makes in its ascent two and one-half spirals, and in our boy who was born deaf it com¬ pleted only one and one-half with the final spiral at the apex missing, and it ended in a large hollow corresponding to the cavity of the final spiral." This is the first description of combined mem¬ branous and bony dysplasia based on an anatomical dissection of the inner was

struck

on

...

ear.

In 1904, Alexander4 described the

histologie features found with this type of malformation, and Sieben¬

scribed by Mondini.79 Characteristi¬ cally, the cochlea is flattened and shortened, with 1 to 1V2 turns instead of the normal 2 V2 to 2% turns. Only the basal turn of the cochlea is fully devel¬ oped; the upper turns form a common cavity known as the scala communis, or the cloaca. The modiolus and os¬ seous spiral lamina are hypoplastic, and the cochlear duct is at times di¬ lated and shortened. The stria vascularis may be atrophied. The organ of Corti is often absent, especially in the upper parts of the cochlea, and the number of spiral ganglion cells are de¬ creased. The endolymphatic duct, ves¬ tibule, and semicircular canals can be enlarged; in extreme cases, the vesti¬ bule has a cystlike appearance (Fig 4). Many patients also have an abnormal stapes. As in cases 2 and 3, there may be an anatomical defect in the foot¬ plate; occasionally, the stapes is totally absent. Patients with Mondini dysplasia are predisposed to developing a CSF leak.

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Fig 4.—Temporal bone section demonstrating typical findings of Mondini malformation.

For this to occur, two abnormalities must exist. On the medial aspect of the labyrinth, CSF must flow quickly from the subarachnoid space into the perilymphatic space. On the lateral aspect of the labyrinth, it must then escape into the middle ear through a defect in the otic capsule. The cochlear aqueduct and the internal auditory canal are two

commonly acknowledged

routes

from the subarachnoid space to the labyrinth. Abnormalities of either can increase CSF flow into the labyrinth. A wide, patent cochlear aqueduct allows CSF to pass directly to the scala tympani, malformed cochlea, and cystlike vestibule. Flow through the internal auditory canal is possible if the lamina cribosa is thin and fragile, as noted in some cases of Mondini dysplasia.9 From the inner ear, CSF can leak through a defect in the otic capsule into the middle ear. The most common site of leakage is the oval window or stapes footplate, although defects have been reported in the round window, eustachian tube, promontory, fallo¬ pian canal, and hypotympanum. Embryologically, Mondini dysplasia results from arrested development during the seventh week of fetal life. Early in the fourth week, the otic placodes, thickened plates of surface ec¬ toderm, appear on either side of the developing hindbrain. As each placode sinks into the underlying mesen-

chyme, an otic pit forms. The edges of each pit fuse to create the otocyst, the precursor of the membranous laby¬

rinth. A hollow diverticulum grows out from this structure and elongates to form the endolymphatic duct and sac. The otocyst then differentiates into a dorsal, or utricular, portion and a ventral, or saccular, portion. Three flat diverticula grow from the utricular portion; their central parts fuse and disappear while the peripheral por¬ tions become the semicircular ducts. This process is complete by the seventh week of gestation. Concomitantly, an outgrowth appears from the ventral saccular portion of the otocyst. It de¬ velops by spiral growth to become the cochlear duct. The duct reaches its full length, 2¥¡ to 2% turns, by the 10th week. The mesenchyme around the otocyst condenses and differentiates into the cartilaginous otic capsule. As the membranous labyrinth enlarges, vac¬ uoles appear in the otic capsule and coalesce to form the perilymphatic space. The cartilaginous otic capsule then ossifies to form the bony laby¬ rinth of the inner ear. Interruption in this orderly progres¬ sion results in a continuum of inner ear malformations. An insult in a 4 week embryo leads to the Michel defect. An arrest at the end of the seventh week leads to a classic Mondini defect, with

the cochlea represented by a short curved tube. The Scheibe and Alex¬ ander defects are the result of devel¬ opmental arrests at later times. The stapedial footplate defect also has a developmental origin. The foot¬ plate consists of two layers: an outer lamina derived from Reichert's carti¬ lage; and an inner lamina, the vestib¬ ular layer, or lamina stapedius, formed from the otic capsule. The initial car¬ tilaginous form of the stapes is re¬ placed by three layers of bone: outer and inner periosteal layers, with an endochondral layer in between. The bone is then remodeled in a resorptive fashion (23 to 26 weeks) to thin the crura and to enlarge the obturator foramen. The vestibular layer of footplate cartilage does not undergo bony re¬ placement, but is preserved as a base on which the outer bony layer of the footplate is deposited. If the vestibular layer of cartilage does resorb, a defect results. The outer layer of bone lacks a cartilaginous base and develops as only a monomeric membrane spanning the breadth of the footplate. A fistula from the vestibule to the middle ear space then results. The child with Mondini malforma¬ tion may present to the otolaryngologist in several ways. If the malforma¬ tion is bilateral, profound sensorineural hearing loss may be the initial sign of a congenital defect. If the malfor¬ mation is unilateral, the dysplasia may go undiagnosed for years. Occa¬ sionally, patients with normal hearing have been reported, but more fre¬ quently, the Mondini ear is clinically unreactive, having neither cochlear nor vestibular function. When a CSF leak develops, find¬ ings depend on the functional status of the involved ear. If there is residual auditory function, a sudden or fluctu¬ ating sensorineural hearing loss may occur. With residual vestibular func¬ tion, the patient may experience ver¬ tigo and have a positive fistula test. A CSF effusion in the middle ear may cause rhinorrhea or otorrhea. More commonly, however, the effusion is asymptomatic and does not cause a perceptible conductive hearing loss, because the ear is nonfunctional. The gravity of the problem than may re-

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main undetected until a myringotomy is performed, releasing a profuse flow of clear fluid (cases 1 and 2). Alterna¬ tively, the CSF effusion may remain undiagnosed until meningitis develops as a result of retrograde infection through the defect in the otic capsule (cases 1 and 3). In addition to the patients described herein, 39 other patients with Mondini dysplasia and meningitis have been described1'2·9" (Table). Age has ranged from 2 months to 41 years, with 60% of patients younger than 4 years and 75% younger than 10 years. No sexual pre¬ dilection occurs. Sixty-seven percent had a unilateral malformation and profound congenital unilateral sensorineural hearing loss. Most had no vestibular symptoms, although formal vestibular testing was rarely per¬ formed. All but three patients suffered at least two episodes of meningitis be¬ fore the correct diagnosis was made, and two patients had 20 episodes of meningitis prior to surgery.34·35 All pa¬ tients required surgical procedures to seal the CSF leak; in 30%, multiple

procedures required to correct the problem. Mondini dysplasia should be sus¬ pected with the clinical triad of con¬ genital (often unilateral) profound sensorineural hearing loss, profound peripheral vestibular weakness, and recurrent meningitis. Associated ab¬ normalities such as Klippel-Feil syn¬ drome, Pendred's syndrome, chromo¬ somal trisomy aberrations, and DiGeorge syndrome may be present, although Mondini dysplasia can result from treatment with teratogenic drugs, like thalidomide, or can occur as an isolated finding. Radiographie studies and brainstem response audiometry confirm the diagnosis. Thin-cut, high-resolution computed tomography is the proce¬ dure of choice; if the presence of a CSF leak is questionable, contrast or radioisotope tracer studies may identify the were

location of the leak. Brain-stem re¬ sponse audiometry should be per¬ formed to determine the functional status of the malformed ear. Surgical intervention is required to avoid further complications. The method of closure of the fistula is dic¬ tated by the functional status of the

Patients With Mondini

Dysplasia

No. of

Source, Biggers

y

al,

et

1973

Barcz et al,'

and

Meningitis*

Stapes footplate

Episodes/ Organism 3/pneumococcus

Sex 2 mo/M

Stapes footplate

5/pneumococcus

8 mo/M

NR

(L)

y/F yr/F

NR

(R)

Site of Lesion

Age, y/

Multiple Audiometry Procedures

1985

Stapes footplate

3/not stated

3

Oval window

3/pneumococcus

7

Carter et

al,'3 1975 Oval window al,37 1978 Oval window

3/pneumococcus

19 mo/M

NR

Clark et

2 / not stated

13

Profound SNHL (R)

Stapes footplate

3/pneumococcus

4

y/F

Profound

Stapes footplate Stapes footplate

4/not stated

1

y/M

SNHL

2/pneumococcus

6 mo/M

Profound SNHL (R)

Oval window

1 / Haemophilus

6

y/M

Profound SNHL (R)

Bottema,12

1975

Crook,"· 1967 Desjardins et al," 1982

y/M

Elverland and

Mair,'e

H influenzae, pneumococcus

(R)

17

y/F

NR

11

y/M

Profound SNHL (L)

Stapes footplate

3/not stated

Stapes footplate

4/not stated

Stapes footplate

20/not stated

7

6 / H influenzae,

10 mo/M

1983

Farrior and

(R)

SNHL (B)

influenzae Oval window

Profound SNHL (L)

(B)

Profound SNHL (B)

Endicott,' 1971

Freeland,3"

1973

y/M

and round

Profound SNHL (R)

window

Guindi,'"

1981

Stapes footplate

Profound

SNHL (R)

pneumococcus

Harrington and Birck," 1967

Oval window and

Harris,1

1978

Oval window

2/pneumococcus

18 mo/M

Profound SNHL (B)

Hicks et

al,20

Stapes footplate

3/not stated

4

Profound SNHL

Stapes footplate

"Multiple"/

1980

Hipskind et al,2' 1976 Hirakawa et al,22 1983 Hirther and

Schindler,23

geniculate ganglion

5/H

influenzae,

4

y/F

pneumococcus

y/not stated

2

y/F

pneumococcus

Profound SNHL (B)

Profound SNHL (R)

y/F

"Deaf-mute"

16 mo/M

Profound SNHL (R)

Stapes footplate Stapes footplate

2/pneumococcus 6/pneumococcus

41

Stapes footplate

4/not stated

3

y/M

NR

(L)

Stapes footplate Stapes footplate

5/not stated

1

(B)

3

y/M y/M

NR

2/pneumococcus

Stapes footplate

3/H influenzae, pneumococcus "Several"/

7

y/F

NR

1985

Kaseff et

al,'

1980

Komune et 1986 Mafee et

al,2s

al,1

Not

reported

1984

Manning and

20

y/F

SNHL (R)

(B)

NR (B)

pneumococcus

Oval window

4/pneumococcus

20 mo/F

Stapes footplate

2/pneumococcus

2

Stapes footplate

2/not stated

13

1/pneumococcus

1

Stapes footplate

3/H influenzae

7

Round window

3/streptococcus

13

SNHL (B)

Gudrun,2' 1983

McNab Jones and Falrburn,! 1977

Mitchell and

Ruben,38 1985

Neely,2

y/M y/F

Profound SNHL (R) NR

(R)

y/M

NR

(R)

y/F y/F

NR

(L)

and round

window 1985

Stapes footplate and round

window Park et

al,

SNHL (R)

1982

(Continued on

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612.)

Patients With Mondini No. of

Source,

Site of

y

Birken,28

Episodes/

Age,

Multiple

y

Lesion_Organism_Sex_Audiometry

Epitympanum Stapes footplate

Pansier and

Dysplasia and Meningitis (cont)*

4

5/pneumococcus 2/pneumococcus

y/M y/M

12

SNHL (B) NR

Procedures 5

(L)

1976_

Schuknecht,9 1980

Schultz and

Stool,3'

Stapes footplate Stapes footplate Stapes footplate

3/pneumococcus 1/pneumococcus 2/pneumococcus

37

NR

27 mo/F

Severe SNHL

Roof of the eustachian

20/not stated

17 mo/not

1

y/F y/M

(R)

...

...

(B)

1970 Stool et 1967

al,35

NR

(R)

stated

tube

y/M

Stapes footplate

4/pneumococcus

10

Stapes footplate

2/pneumococcus

27 mo/F

Profound

2

_SNHL (L)_ Tschiang et al,32

NR

(L)

1973_

Vermeersch et

Stapes footplate

al,33

29 mo/M

31H influenzae

NR

()

1980_ Stapes footplate

Present case

*NR

3

indicates

no

response; L, left ear; R,

right

ear;

hearing is present, it may be possible to stop the leak with a fasciai graft. If a stapedectomy is performed, a stapes gusher may occur and pre¬ ear.

If

clude a favorable outcome. When the ear is nonfunctional, the vestibule should be packed with fascia, muscle, or fat, and the oval window corked with bone. Alternatively, the middle ear cavity can be obliterated.36 If the initial procedure does not effectively seal the CSF leak, repeated surgical procedures become necessary. Occa¬ sionally, an intracranial approach is required to pack the meatus of the in¬ ternal auditory canal or the cochlear

aqueduct.35·37·38 CONCLUSIONS

The child with a Mondini dysplasia is at risk not only for hearing impair¬ ment, but also for recurrent meningi¬ tis. The diagnosis of the inner ear ab¬ normality requires thin-cut computed tomographic scanning. Audiometrie testing dictates the surgical approach necessary to correct the problem. Hearing preservation would be ideal, but the primary goal of surgery must be to stop the CSF leak. References 1. Harris H. Cerebrospinal otorrhea and recurring meningitis: report of three cases. Laryngoscope.

1978;88:1577-1585.

15 mo/M

2/pneumococcus

Profound SNHL (L)

SNHL, sensorineural hearing loss; and B, both

ears.

Neely JG. Classification of spontaneous cerebrospinal fluid middle ear effusion: review of 49 cases. Otolaryngol Head Neck Surg. 1985;93:625\x=req-\ 2.

634. 3. Mondini C. Anatomia surdi nedi sectio: Dr Bononiensi Scientiarum et Artium Instituto Atque Academia Commentorii. Boniesi. 1791;7:28, 419. 4. Alexander G. Zur Pathologie und pathologiochen Anatomie der Kongenitalen Taub heit. Arch Klin Exp Ohren Nasen Kehlkopfheilkd.

1904;61:183.

5. Siebenmann F. Grundzuge der Anatomie und Pathogeneses der Taubstummheit. Wiesbaden, West Germany: JF Bergmann; 1904. 6. Fraser JS. A case of congenital deafness with malformation of the bony and membranous labyrinths on both sides. Proc R Soc Med. 1927; 20:475-477. 7. Igarashi M, Filippone MV, Alford BR. Temporal bone findings in Pierre Robin syndrome.

Laryngoscope. 1976;86:1679-1687. 8. Paparella MM. Mondini's deafness: a review of histopathology. Ann Otol Rhinol Laryngol Suppl. 1980;89 (suppl 67):1-10. 9. Schuknecht HF. Mondini dysplasia: a clinical and pathological study. Ann Otol Rhinol Laryngol Suppl. 1980;89 (suppl 65):1-23. 10. Biggers P, Howell NN, Fischer ND, Himadi GM. Congenital ear anomalies associated with otic meningitis. Arch Otolaryngol. 1973;97:399\x=req-\ 401. 11. Barcz D, Wood RP, Stears J, Jafek BW, Shields M. Subarachnoid space: middle ear pathways and recurrent meningitis. Am J Otol.

1985;6:157-163.

12. Bottema T. Spontaneous cerebrospinal fluid otorrhea. Arch Otolaryngol. 1975;101:693\x=req-\ 694. 13. Carter BL, Wolpert SM, Karmody CS. Recurrent meningitis associated with an anomaly of the inner ear. Neuroradiology. 1975;9:55-61. 14. Crook JP. Congenital fistula in the stapedial footplate. South Med J. 1967;60:1168-1179. 15. Desjardins R, Guergverian AJ, DubeJ, Deschamps N, Lavertu P. Meningitis and congenital fistula of the internal ear. J Otolaryngol. 1982;

11:97-100. 16. Elverland HH, Mair IWS. Recurrent meningitis, congenital anacusis and Mondini anomaly. Acta Otolaryngol (Stockh). 1983;95:147-151. 17. Farrior JB, Endicott JN. Congenital mixed deafness: cerebrospinal fluid otorrhea, ablation of the aqueduct of the cochlea. Laryngoscope. 1971; 87:684-699. EM. Congenital labyrintho18. Guindi tympanic fistula: a recently recognized entity in chiledren. J Otolaryngol. 1981;10:67-71. 19. Harrington JW, Birck HG. Recurrent meningitis due to congenital petrous fistula. Arch

Otolaryngol. 1967;85:128-131. 20. Hicks GW, Wright JW Jr, Wright JW III. Cerebrospinal fluid otorrhea. Laryngoscope. 1980; 90(suppl 25):1-25. 21. Hipskind MM. Lindsay JR, Jones TD, Valvassori GE. Recurrent meningitis and labyrinthine gusher, related to congenital defects of the labyrinthine capsule and stapes footplate. Laryngoscope 1976;86:682-689. 22. Hirakawa K, Kurokawa M, Yajin K, Haranda Y. Recurrent meningitis due to a congenital fistula in the stapedial footplate. Arch Otolaryngol. 1983;109:697-700. 23. Hirther C, Schindler RA. Mondini's dysplasia with recurrent meningitis. Laryngoscope. 1985;95:655-658. 24. Kaseff LG, Nieberding PH, Shorago GW,

Huertas G. Fistula between the middle ear and subarachnoid space as a cause of recurrent meningitis: detection by means of thin-section, com-

plex-motion tomography. Radiology. 1980;135:

105-108. 25. Komune S, Enatsu K, Morimitsu T. Recurrent meningitis due to spontaneous cerebrospinal fluid otorrhea. Int J Pediatr Otorhinolaryngol.

1986;11:257-264. 26. Mafee MF, Selis JE, Yannias DA, et al. Congenital sensorineural hearing loss. Radiology. 1984;150:427-434. 27. Manning KP, Gudrun R. Recurrent meningitis secondary to concealed cerebrospinal fluid otorrhea. Arch Dis Child. 1983;11:153-155. 28. McNab Jones RF, Fairburn B. Spontaneous cerebrospinal fluid otorrhoea. J Laryngol Otol. 1977;91:897-902. 29. Parisier SC, Birken EA. Recurrent meningitis secondary to idiopathic oval window CSF leak. Laryngoscope. 1976;86:1502-1515. 30. Park TS, Hoffman HJ, Humphreys RP, Chuang SH. Spontaneous cerebrospinal fluid otorrhea in association with a congenital defect of the cochlear aqueduct and Mondini dysplasia. Neurosurgery. 1982;11:356-362. 31. Schultz P, Stool S. Recurrent meningitis due to a congenital fistula through the stapes footplate. AJDC. 1970;120:553-554. 32. Tschiang HH, Harrison MS, Ozsahinaglu CA. Cerebrospinal otorrhoea. J Laryngol. 1973; 87:457-483. 33. Vermeersch M, Kluyskens P, Vanderstock L. The temporal bone as route of infection in recurrent meningitis. J Otolaryngol. 1980;9:199-201. 34. Freeland AP. Non-traumatic CSF rhinorrhoea associated with congenitally malformed ears. J Laryngol Otol. 1973;87:781-786. 35. Stool S, Leeds NE, Shulman K. The syndrome of congenital deafness and otic meningitis: diagnosis and management. J Pediatr. 1967; 71:547-552. 36. Schuknecht HF, Chandler JR. Surgical obliteration of the tympanomastoid compartment and external auditory canal. Ann Otol Rhinol

Laryngol. 1984;93:641-645.

37. Clark JL, Desanto LW, Facer GW. Congenital deafness and spontaneous CSF otorrhea. Arch

Otolaryngol. 1978;104:163-166.

38. Mitchell BP, Ruben AM. Mondini dysplasia: late complications. J Otolaryngol. 1985;14:265-267.

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