Cochlear Aplasia- A Rare Case

Abstract

(M.P.) HOW TO CITE THIS ARTICLE: Agrawal K, Rathore RK, Chauhan A, et al. Cochlear aplasia-a rare case. J. Evolution Med. Dent. Sci. 2017;6(95):7063-7065, DOI: 10.14260/jemds/2017/1533 PRESENTATION OF CASE To report a rare case of cochlear aplasia in a 23-year-old male with profound unilateral hearing loss for more than 10 years without a definitive diagnosis. In October 2016, a 23-year-old male patient came to CRG Hospital ENT OPD with chief complaint of decreased hearing in left ear for past 2 months. As per the patient, he was experiencing hearing loss from left ear for more than 10 years, but for the last two months the patient started having difficulty in hearing more from left ear than right ear, which was more profound when patient was using mobile phones or headphones. Patient has no complaints of tinnitus, headache or discharge from B/L ears, vomiting, vertigo, chronic systemic illness like DM, HTN or asthma.Patient gives no similar family history. On physical examination patient was awake, conscious and well oriented to time, place and person with normal blood pressure and pulse with no icterus, pallor, cyanosis, clubbing, cervical lymphadenopathy, organomegaly or oedema. On ear examination, bilateral ears had normal external auditory canal with intact and normal tympanic membranes.Examination of the nasal cavity revealed deviated nasal septum towards right side. Throat examination was normal with normal vocal cords on indirect laryngoscopy. On examination by tuning fork tests, no perception of sound is noted in the left ear on absolute bone conduction test and Rinne's test; however, Weber is lateralised to the right ear. PATHOLOGICAL DISCUSSIONCochlear aplasia or complete absence of the cochlea is most likely due to arrested development of the inner ear in the later part of the 3rd week of gestation or as described by some in the 5th week. It is a rare anomaly accounting for only 3% of cochlear malformations. 1 In cochlear aplasia the vestibule and semicircular canals are often malformed but may be normal. 2 Dense otic bone is present at the site where the cochlea normally would be and is best depicted on CT images. It is important to differentiate this anomaly from labyrinthitis ossificans, in which normalised bone is seen anterior to the IAC with the bulge of the cochlear promontory produced by the basal turn of the cochlea, both features are absent in cochlear aplasia. 'Financial or Other Competing Interest': None. Submission 12-10-2017, Peer Review 25-11-2017, Acceptance 01-12-2017, Published 23-12-2017. Corresponding Author: Rajesh Kumar, Flat no 88, Amba Enclave, Sector 9 Rohini, Delhi-110085. E-mail: tokasrajesh@gmail.com DOI: 10.14260/jemds/2017/1533 We report one such rare case of a 23-year-old male who had unilateral profound hearing loss for more than 10 years and has been investigated several times, but no definitive diagnosis was made. Physical and ear examination were normal and tuning fork tests and audiogram show sensorineural type of unilateral hearing loss. 2 When the pa...