Abstract
BackgroundAutoimmune 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.MethodsOver 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.ResultsSixty-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).ConclusionAIED 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.
Highlights
Autoimmune inner ear disease (AIED) is a rare condition characterized by bilateral fluctuating sensorineural hearing loss (SNHL)
Sixty-four automated home audiograms and nine routine clinical audiograms were conducted over a 9-month period using the tablet audiometer device
On 28 occasions the pure tone average (PTA) varied by at least 5 dB on a subsequent test occurring within 4 days of the previous (Table 1), and on 6 occasions the PTA fluctuated by more than 15 dB within 24 h. 5 dB was chosen as the limit for significance, as this is widely felt to be the test-retest threshold for clinical pure-tone audiometry [15]
Summary
Autoimmune inner ear disease (AIED) is a rare condition characterized by bilateral fluctuating sensorineural hearing loss (SNHL). While conventional sound booth audiograms are the gold standard for the assessment of a patient’s hearing, they are relatively time consuming, require the expertise of a trained audiologist, and provide only a brief snapshot of the patient’s hearing While this does not pose a problem for most forms of hearing loss, it can be difficult to adequately assess and treat a AIED can be classified as primary AIED (immune mediated disease limited to the inner ear) or secondary AIED (immune mediated inner ear disease as a manifestation of a systemic autoimmune process) [3]. Costly and resource intensive, plasmapheresis has been suggested as an adjunct to steroids or cytologic agents for patients with refractory disease [4], and has even been shown to allow as many as 75% of patients to wean from immunosuppression [8] Biologic agents such as Etanercept and Rituximab have been studied with conflicting results [9, 10].
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