[Skip to Content]
[Skip to Content Landing]
Citations 0
Clinical Problem Solving: Radiology
October 2002

Radiology Quiz Case 2

Author Affiliations
 

R. NICKBRYANMDS. JAMESZINREICHMD

Arch Otolaryngol Head Neck Surg. 2002;128(10):1214. doi:

A 51-YEAR-OLD WOMAN presented with a history of otosclerosis and bilateral stapedectomies that she had undergone approximately 20 years earlier, with minimal benefit. Since then, she experienced a slow deterioration of hearing in both ears. She noted no family history of otosclerosis. Otoscopic examination revealed normal, mobile tympanic membranes in both ears. A Weber test demonstrated localization to the left ear, and the results of Rinne tuning fork tests were equivocal. The patient's audiogram revealed severe to profound sensorineural loss in the left ear, with thresholds ranging from 70 dB at 250 Hz to 105 dB at 4000 Hz and no response at 6000 or 8000 Hz. The right ear demonstrated no response. Speech awareness at 85 dB was observed for the left ear only. Tympanograms were type A bilaterally. Acoustic reflexes were absent.

Speech perception testing under best-aided binaural condition (at 57-dB hearing level in sound field) revealed CNC (consonant-nucleus-consonant) word recognition of 16%; CNC phonemes, 29%; HINT (Hearing in Noise Test)-quiet, 61%; HINT-noise, 34%; and everyday sentences, 61%. As the patient had excellent speechreading skills and appropriate expectations, cochlear implantation was pursued.

Right transmastoid cochlear implantation was performed in a routine manner. A posterior tympanotomy and identification of the round window and oval niche were performed. There was no visible stapes. The piston prosthesis could be identified within the oval window niche, and the bone on the promontory appeared more prominent than usual. Otospongiotic bone and hypervascular bone were encountered within the round window niche. Nevertheless, cochleostomy into the basal turn was feasible. The caliber of the scala tympani was smaller than usual, with dense bone present at the level of the cochlear endosteum. On further enlargement of the cochleostomy, a steady flow of clear fluid developed, which resolved over 1 minute of observation.

An electrode array (Nucleus-24; Cochlear Corp, Englewood, Colo) was implanted through the cochleostomy. Full insertion of the array was achieved. One month later, and despite aggressive programming, the patient detected no sound. Facial stimulation was also noted to occur with activation of several electrodes. Transorbital plain films (Figure 1) and computed tomograms (CTs) of temporal bone (Figure 2) were obtained to verify the position of the cochlear implant.

What is your diagnosis?

×