Mobile tablet audiometry in fluctuating autoimmune ear disease

AIED is a rare and relatively poorly understood condition that can lead to profound hearing loss and significant patient morbidity [2, 4–6, 16]. It is seemingly more prevalent in women, and most commonly presents in the third to sixth decade of life [2]. While patients will often present with unilateral hearing loss, approximately 80% patients will eventually develop bilateral disease [2, 6] (although the severity of the loss is often asymmetric [7, 17, 18]). Up to 50–80% of patients will develop associated otologic symptoms including tinnitus and aural fullness [4, 6] and the incidence of associated vestibular symptoms is as high as 79% in the literature [6]. These associated symptoms will often fluctuate along with the hearing [4]. The hearing loss and associated symptoms often worsen progressively over a period of weeks to months, although sudden deafness or severe vestibular hypofunction has been reported [19]. The prognosis is usually poor with progression to severe losses by the age of 30.

The patient described herein reported fluctuating hearing at home, however it was unclear to the treating team the range and rapidity of the variability. This presented as difficulty using standard amplification and problems with school participation. Our study has demonstrated the highly fluctuating nature of AIED, and has shown that regular home audiograms can be a feasible and reliable way to monitor a patient’s clinical progression over time. In this patient’s case standard intratympanic steroid therapy was ineffective over several months. We were initially hesitant to proceed with a cochlear implant given that the patient still had hearing in one ear. However, we were unaware of the severity and frequency of their problems until daily monitoring was undertaken. As a result they received the implant in the contralateral ear much sooner than would otherwise occurred, with the knowledge that some days the patient will have two functional ears and other days not.

We propose that clinicians could use home audiometry devices for a wide array of uses in the AIED patient population. Tablet audiometry can be used to document patients’ progression over time, identify major fluctuations earlier in the disease process, as well as monitor their response to treatment. The benefit of corticosteroid therapy for AIED has been shown to wane over time [7], and earlier detection of recalcitrant hearing loss may allow specialists to initiate advanced treatment sooner. Data-driven therapy has the potential to decrease not only the patient morbidity associated with worsening hearing loss, but that associated with treatment side effects. Corticosteroids are associated with a long list of adverse side effects including weight gain, diabetes, immunosuppression, osteoporosis and avascular necrosis of the femoral head. By using regular audiogram data to identify the point where corticosteroid therapy has outlived its clinical benefit, physicians can discontinue therapy as soon as it becomes ineffective, reducing the risk of adverse events associated with the medication.

Access to home audiometry may assist patients with their rehabilitation as patients with fluctuating losses complain of limited utility of hearing aids due to the constantly changing fit. Having access to daily home audiometry results may enable patients to more accurately refit their hearing aids in response to fluctuations in their hearing throughout the week. Already, many modern hearing aids allow patients to manually select programs on their hearing aids using an app on their mobile phone. Further, new models for the sale and fitting of hearing aids are emerging with the advent of ‘Self-fit hearing aids over-the-Internet’ and ‘Personal Sound Amplification Products’ (PSAPs). Indeed, there is a new trend in medicine of increased patient generated data and patient ownership of that data. In order to ensure reliable results and responsible care of the patient the medical community must participate in this evolution or risk being supplanted. However, several barriers to this method due exist, including access to appropriate home equipment, access to suitably quiet environments and the level of training required to administer and interpret results. Previous research has demonstrated that un-calibrated headphones or ear-buds do not provide reliable results and that there is great variability between different types of home hearing tests in terms of accuracy and ability to handle background noise, however, calibrated systems can perform well in suitable environments. [20–22]. There is also still debate about who is appropriate to be tested at home and who is capable of performing the test. Nevertheless, with the burgeoning of on-line training, gamified testing and artificial intelligence diagnostic support we see a path where future research and development could greatly improve the efficacy of testing and rehabilitation in this challenging population.

While our study only investigated one patient with AIED, further work could be done to collect regular audiometry data from a larger cohort of patients with AIED or other conditions such as Meniere’s disease or examine the speed of patient hearing recovery from surgery. Patient generated data appears to be useful, informative and satisfying to patients in other areas of medicine and this may be similar.