Mobile tablet audiometry in fluctuating autoimmune ear disease

BACKGROUND: Autoimmune inner ear disease (AIED) is a rare condition characterized by bilateral fluctuating sensorineural hearing loss (SNHL). The labile nature of this hearing loss makes it difficult to accurately quantify with conventional methods, and therefore it is challenging to rehabilitate. METHODS: Over a 9-month period one pediatric patient with severe AIED was monitored and conducted home audiograms using a previously validated testing system (Shoebox Audiometry). During this period he also underwent several clinical audiograms. The correlation between clinical and home audiograms was analyzed with a Pearson coefficient, and the range and frequency of fluctuations was recorded. RESULTS: Sixty-four automated home audiograms and nine clinical audiograms were conducted. When tested at home using a calibrated system the pure tone average (PTA) fluctuated between 12 dB and 72 dB indicating large variability in hearing. Fluctuations were frequent: on 28 occasions the PTA varied by at least 5 dB when retested within 4 days. The mean PTA was 50 dB and 95% of the thresholds were within 36 dB of the mean. Clinical audiograms obtained on the same day or within 1 day of home testing were highly concordant (with a Pearson coefficient of 0.93). CONCLUSION: AIED can result in significant fluctuations in hearing over short periods of time. Home testing enables a more granular look at variations over time and correlates well with clinical testing, and thus facilitates rapid action and informed rehabilitation. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s40463-017-0195-1) contains supplementary material, which is available to authorized users.