A Study of Middle Ear Diseases in Children with Congenital Bilateral Severe to Profound Sensorineural Hearing Loss.

(1) To find the presence of middle ear diseases present in the patients with congenital bilateral severe to profound SNHL (2) If it poses as a threat to rehabilitative efforts for the existing severe to profound SNHL. Study setting: Department of ENT, C U Shah medical college and hospital. Study design: Prospective study Study population: Patients attending ENT department with complaints of congenital bilateral severe to profound sensorineural hearing loss. A total of 50 cases were studied during the study period. Methods of data collection: The Proforma was designed based on objective of the study. Detailed history was taken followed by thorough ENT and systemic examinations. Otoscopy and otoendoscopy were carried out and all patients were subjected to hearing tests consisting of Pure Tone Audiometry (PTA), Impedance Audiometry (IA), Brainstem Evoked Response Audiometry (BERA) and Oto Acoustic Emissions (OAE). HRCT and MRI scanning of temporal bones of all the patients was included as a part of the routine workup. Out of the 50 children with bilateral congenital hearing loss studied in this study, 13(26%) children were observed to have concurrent middle ear pathologies. 2 patients had bilateral retracted drum with tympanosclerosis; 3 had bilateral retraction pocketswith mastoiditis; 1 had bilateral SOM; 1 had right SOM and left retracted drum; 2 had bilateral PSQ cholesteatoma; 1 had left SOM and right sided tympanic membrane perforation, 3 had right sided tympanic membrane perforation with left sided normal ears. Children with congenital bilateral severe to profound hearing loss should be examined for middle ear pathologies, which can most often be overlooked otherwise, hence rendering the patient unfit for definitive management of the severe to profound SNHL in the form of Hearing Aid trial or Cochlear Implantation, further delaying the development of speech. Hence, all children with congenital bilateral severe to profound hearing loss should undergo regular screening for assessment of middle ear pathologies with prompt treatment when any middle ear pathology is encountered, therefore rendering the patient fit for fitting of cochlear implantation at the earliest possible to decrease permanent impairment of speech.