Oval window perilymph fistula in child with recurrent meningitis and unilateral hearing loss

2020 ◽  
Vol 13 (7) ◽  
pp. e234744
Author(s):  
Miane Min Yan Ng ◽  
Felice D'Arco ◽  
Raouf Chorbachi ◽  
Robert Nash

A 2-year-old boy presented to Ears, Nose and Throat (ENT) surgeons with unilateral hearing loss. Following a prodrome of upper respiratory tract infection (URTI), he developed two episodes of pneumococcal meningitis in quick succession. This case demonstrates an unusual cause of perilymph fistula diagnosed on imaging and confirmed surgically. He had failed the Newborn Hearing Screening Programme and was therefore referred to audiology, who confirmed profound sensorineural hearing loss in the right ear. MRI showed incomplete partitioning (type 1) of the right cochlea, suggesting cerebrospinal fluid (CSF) leak from the region of the stapes. Exploratory tympanotomy confirmed this, and proceeded to CSF leak repair, obliteration of the Eustachian tube, subtotal petrosectomy, abdominal fat grafting and blind sac closure. Although middle ear effusions are common; particularly in children with recent URTI, the possibility of otogenic CSF leak needs to be considered, especially in cases of recurrent meningitis.

2001 ◽  
Vol 115 (2) ◽  
pp. 132-135 ◽  
Author(s):  
Gregor Bachmann ◽  
Marco Nekic ◽  
Olaf Michel

The incidence of perilymphatic fistula as cause of sudden hearing loss is not known. We present a case with sudden unilateral hearing loss associated with a positive β-trace protein test of an epipharyngeal fluid sample. The patient presented with sudden sensorineural hearing loss on the right side. A stapedotomy had been performed nine months previously due to otosclerosis. Intravenous therapy for the treatment of sudden hearing loss was unsuccessful. At the time of sudden hearing loss, epipharyngeal fluid was collected using a Raucocel sinus pack. Investigation using rocket immunoelectrophoresis showed the presence of β-trace protein. Upon repeating tympanoscopy there was no obvious labyrinthine fluid egress, but the oval window was sealed with fibrin sponge and fibrin glue. The patient’s hearing improved over a period of five months.


Author(s):  
Kiran Natarajan ◽  
Koka Madhav ◽  
A. V. Saraswathi ◽  
Mohan Kameswaran

<p>Bilateral temporal bone fractures are rare; accounting for 9% to 20% of cases of temporal bone fractures. Clinical manifestations include hearing loss, facial paralysis, CSF otorhinorrhea and dizziness. This is a case report of a patient who presented with bilateral temporal bone fractures. This is a report of a 23-yr-old male who sustained bilateral temporal bone fractures and presented 18 days later with complaints of watery discharge from left ear and nose, bilateral profound hearing loss and facial weakness on the right side. Pure tone audiometry revealed bilateral profound sensori-neural hearing loss. CT temporal bones &amp; MRI scans of brain were done to assess the extent of injuries. The patient underwent left CSF otorrhea repair, as the CSF leak was active and not responding to conservative management. One week later, the patient underwent right facial nerve decompression. The patient could not afford a cochlear implant (CI) in the right ear at the same sitting, however, implantation was advised as soon as possible because of the risk of cochlear ossification. The transcochlear approach was used to seal the CSF leak from the oval and round windows on the left side. The facial nerve was decompressed on the right side. The House-Brackmann grade improved from Grade V to grade III at last follow-up. Patients with bilateral temporal bone fractures require prompt assessment and management to decrease the risk of complications such as meningitis, permanent facial paralysis or hearing loss. </p>


2015 ◽  
Vol 30 (2) ◽  
pp. 56-58
Author(s):  
Ryner Jose D. Carrillo ◽  
Precious Eunice R. Grullo ◽  
Maria Luz M. San Agustin

Dear Editor,   The tympanic membrane and the ossicular chain contribute roughly 28 dB in hearing gain. In chronic suppurative otitis media, loss of tympanic membrane and lysis of the ossicular chain are significant causes of hearing loss.1 Through the years, hearing impairment has been augmented using various devices such as ear trumpets, carbon hearing aids, vacuum tube and transistor hearing aids, bone anchored hearing aids, and cochlear implants.2 This case report describes how a cotton wick was used to amplify sound.   Case Report A 65-year-old man consulted for hearing loss. He had a childhood history of recurrent ear discharge and hearing loss and was diagnosed with chronic suppurative otitis media. At age 55, he underwent tympanomastoidectomy of the left ear. While surgery stopped the left ear discharge, there was complete hearing loss in this ear. For this reason, he opted not to have surgery on the right ear. There was subsequent recurrent ear disease of the right ear. He would clean his ear with a cotton wick and apply antibiotic drops during bouts of ear discharge. He observed that leaving the ear wick with a few drops of topical otic preparations (polymyxin-neomycin-steroid or ofloxacin) would lessen the frequency of ear discharge and improve his hearing.  He found that morning application and positioning of the cotton wick in his right ear using tweezers and a toothpick allowed him to hear adequately to conduct his daily activities as an architect. (Figure 1, 2) The fear of hearing loss from another surgery, cost of a commercial hearing aid, and great utility of a simple cotton wick made him continue his practice for these ten years. Examination of the right middle ear without the cotton wick showed thickened mucosa, absent malleus and incus structures, a patent Eustachian tube and a near – total tympanic membrane perforation. There was no keratinous material or foul smelling discharge. (Figure  3) Pure tone audiometry confirmed that with the cotton wick, the right air-bone gap decreased at 500 hz, 1kHz, 2Kh and 4KHz by 30db, 40dB, 35dB and 25dB respectively.  (Table 1) DISCUSSION At different anatomic levels, mechanical sound energy is amplified and transmitted to the functional parts of the ear. The tympanic membrane and oval window ratio of 21:1 and malleus-incus lever mechanism ratio of 1.3:1 provide a 28 dB amplification of conductive hearing.1 This gain is reflected by frequency specific air-bone gaps, which can range between 25-40 dB. With the contribution from the external ear, the overall conductive gain is 60 dB.1,3 Damage to the auditory system often results in a loss of hearing sensitivity that is frequency – specific.  The presence of a frequency – specific wide air-bone gap suggests ossicular chain discontinuity among patients with chronic otitis media.4 Narrowing of the air-bone gap, which in this case was provided by insertion of the cotton wick, may lead to at least partial restoration of ossicular coupling. The ability of the cotton wick to improve hearing may be attributed to its possession of characteristics for sound conduction and acoustic impedance, such as stiffness, resistance and mass.  The effectiveness of the cotton wick was reported to be dependent on its positioning in the ear; the patient would have to insert the wick down to the level of the promontory or oval window, occasionally blow his nose, or reposition the cotton wick to achieve an acceptable hearing level. However, for a patient with completely deaf contralateral ear, a 32.5 dB gain in hearing is very pronounced and significant.  The hearing gain produced by the cotton wick only amplified the air conductive component of hearing but not bone conduction. While it afforded amplification of sound and a route of medicine administration, it may also have contributed to sensorineural hearing loss brought about by ototoxicity of medications and thickening of the oval and round window from chronic irritation. For this reason, utmost caution must be advised before considering use of a “cotton wick” to amplify hearing in this manner-- a practice we do not endorse. The cotton wick may have served as a vibrating piston on top of the oval window which amplified hearing. Such a mechanism may conceivably prognosticate potential gain from a contemplated tympanoplasty in the same way that the “paper patch test”5 predicts simple myringoplasty outcomes. Having said that, the diagnostic utility of such a cotton wick requires further investigation before potential clinical applications such as prognostication of tympanoplasty are theorized. Could future studies show that a preoperative cotton wick (or equivalent device) may approximate potential gains from a good tympanoplasty with ossiculoplasty in a patient with total tympanic perforation and ossicular chain loss?   Sincerely, Ryner Jose D. Carrillo, MD, MSc Precious Eunice R.  Grullo, MD, MPH Maria Luz M. San Agustin, RN, MClinAudio    


1989 ◽  
Vol 103 (5) ◽  
pp. 473-480 ◽  
Author(s):  
R. E. Quiney ◽  
D. B. Mitchell ◽  
B. Djazeri ◽  
J. N. G. Evans

AbstractRecurrent meningitis in children is not only a potentially life threatening condition, but often involves the child in the trauma of repeated hospital admissions and multiple and invasive investigations to try and find an underlying cause. Symptoms and signs of CSF rhinorrhoea or otorrhoea are infrequent in these patients. Unilateral deafness may be difficult to diagnose in the young child. Full ENT examination may be normal.We report seven cases of children with recurrent meningitis in whom inner ear abnormalities were only indicated as the site of entry of infection by hypocycloidal tomography or high resolution CT scanning of the temporal bone. Subsequent tympanotomy confirmed the site of the CSF leak as the oval window in the majority of cases; packing the vestibule with muscle halted further attacks in these patients.


1993 ◽  
Vol 107 (8) ◽  
pp. 726-729 ◽  
Author(s):  
D. S. Stevenson ◽  
D. W. Proops ◽  
P. D. Phelps

AbstarctMeningitis may be the sole presenting sign of a cerebrospinal fluid (CSF) fistula of the temporal bone. An eight-year-old boy suffering from recurrent meningits was found to hav ebilateral severe cochlear dysplasia. Bilateral tympanotomies were performed, planning to obliterate each vestibule. In the right ear a stapedectomy was oerfirned, resulting in terrntial ‘CSF gusher’ and difficulty in packing the vestibule. CSF rhinorrhoea requiring revision surgery and twon episodes of gram-negative bacterial meningtis complicated the post-operative management, resulting in a prolonged hospital stay. Subsequently, the left ear was managed in a different fasion, leaving the stapes in situ, with grafts placed to seal the oval window nche. We would recommended this alternative procedure in cases of severe cochlear dysplasia, where abnormalities of the vestibule and basal turn of the cochela mean that performing a stapeddectomy to pack the vestibule may result in a severe ‘CSF gusher’, by opening directly into the subarachnoid space.


2013 ◽  
Vol 40 (3) ◽  
pp. 251-259 ◽  
Author(s):  
Gülsüm Aydan Genç ◽  
Özlem Konukseven ◽  
Nuray Bayar Muluk ◽  
Günay Kirkim ◽  
Figen Suren Başar ◽  
...  

2020 ◽  
Vol 51 (1) ◽  
pp. 98-102
Author(s):  
Dawna E. Lewis

Purpose This epilogue discusses messages that we can take forward from the articles in the forum. A common theme throughout the forum is the ongoing need for research. The forum begins with evidence of potential progressive hearing loss in infants with mild bilateral hearing loss, who may be missed by current newborn hearing screening protocols, and supports the need for consensus regarding early identification in this population. Consensus regarding management similarly is a continuing need. Three studies add to the growing body of evidence that children with mild bilateral or unilateral hearing loss are at risk for difficulties in speech understanding in adverse environments, as well as delays in language and cognition, and that difficulties may persist beyond early childhood. Ambivalence regarding if and when children with mild bilateral or unilateral hearing loss should be fitted with personal amplification also impacts management decisions. Two articles address current evidence and support the need for further research into factors influencing decisions regarding amplification in these populations. A third article examines new criteria to determine hearing aid candidacy in children with mild hearing loss. The final contribution in this forum discusses listening-related fatigue in children with unilateral hearing loss. The absence of research specific to this population is evidence for the need for further investigation. Ongoing research that addresses difficulties experienced by children with mild bilateral and unilateral hearing loss and potential management options can help guide us toward interventions that are specific for the needs of these children.


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