Estudio de la audición (128-20.000 Hz) en mujeres con tiroiditis autoinmune: papel de los anticuerpos frente al déficit de hormona tiroidea / Audiological evaluation (128-20.000 Hz) in women with autoinmune thyroiditis: The role of antibodies vs. l-thyroxine deficiency

Antecedentes y objetivo: Hay muchas causas de hipoacusia neurosensorial, entre las que tiene especial interés la patología autoinmune del oído interno por su posible reversibilidad con tratamiento esteroideo, a veces asociada a enfermedades sistémicas autoinmunes. La tiroiditis crónica autoinmune o de Hashimoto (TA) presenta anticuerpos que podrían afectar al oído interno independientemente del hipotiroidismo, efecto que no ha sido suficientemente estudiado y nunca mediante audiometría con extensión en altas frecuencias.El objetivo de este trabajo es estudiar si existe afectación de los umbrales auditivos en pacientes de TA, sin hipotiroidismo, en la totalidad del espectro auditivo humano (128 Hz – 20 kHz) distribuido por grupos de edad.Materiales y métodosSe han seleccionado 128 pacientes divididos en dos grupos. El primer grupo de pacientes presenta anticuerpos antitiroideos elevados sin necesitar tratamiento sustitutivo con tiroxina. El segundo grupo con tratamiento sustitutivo con tiroxina, bien controlados. Se comparan con el grupo control (GC) de 209 pacientes. En todos se realizó historia clínica, exploración otológica, estudio de niveles de anticuerpos antitiroideos, TSH (thyroid-stimulating hormone), T3 y T4 libres, timpanograma, estudio audiométrico convencional y con extensión en altas frecuencias.ResultadosTodos los pacientes fueron mujeres. Ambos grupos mostraron peor audición que los controles, siendo la diferencia estadísticamente significativa en todas las frecuencias; en el rango de frecuencias de 8 – 20 kHz con una diferencia de más de 10 dB, y en los rangos de 9-16 kHz y de 20 kHz de más de 20 dB. (AU)

Background and aim: Although sensorineural hearing loss may have different aetiologies, we focused on autoimmune hearing loss since it may be reversible with corticosteroid therapy; this entity is sometimes associated with systemic autoimmune diseases. Hashimoto's thyroiditis or chronic autoimmune thyroiditis shows antibodies and may be harmful to hearing thresholds regardless of hypothyroidism effect. To date this effect has not been sufficiently studied and never with extended high frequencies. The aim of this work is to study by age groups whether hearing thresholds in the human auditory range (128 to 20.000 Hz) are affected in Hashimoto's disease.Materials and methodsTwo groups of 128 patients affected by Hashimoto's thyroiditis were included. First group: patients with pathological antithyroid antibodies who do not need L-thyroxine treatment. Second group: patients controlled with L-thyroxine substitutive treatment. Audiometric threshold study comparing between the groups of patients and a group of 209 controls was performed. All patients underwent complete otorhinolaryngological examination, antithyroid antibodies, TSH, T3 and T4 blood levels, tympanometry, conventional pure-tone audiometry, and extended-high-frequency audiometry.ResultsAll patients were women. Both groups showed worst audiometric thresholds than the control group; both study groups showed worse hearing than controls, this difference was statistically significant in all frequencies. In the 8-20 kHz frequency range this difference was more than 10 dB, and in the 9-16 kHz and 20 kHz range this difference was more than 20 dB. When separated by age groups, in younger subjects (20-29 years) these differences were found in all frequencies, except for conversational frequencies (500 - 4,000 Hz); between 30 and 49 years the difference is statistically significant in all frequencies; and from 50 to 69 years differences are found, especially in the conversational frequencies. (AU)

Audiological evaluation (128-20,000Hz) in women with autoimmune thyroiditis: The role of antibodies vs. l-thyroxine deficiency.

BACKGROUND AND AIM: Although sensorineural hearing loss may have different aetiologies, we focused on autoimmune hearing loss since it may be reversible with corticosteroid therapy; this entity is sometimes associated with systemic autoimmune diseases. Hashimoto's thyroiditis or chronic autoimmune thyroiditis shows antibodies and may be harmful to hearing thresholds regardless of hypothyroidism effect. To date this effect has not been sufficiently studied and never with extended high frequencies. The aim of this work is to study by age groups whether hearing thresholds in the human auditory range (128-20,000Hz) are affected in Hashimoto's disease. MATERIALS AND METHODS: Two groups of 128 patients affected by Hashimoto's thyroiditis were included. First group: patients with pathological antithyroid antibodies who do not need L-thyroxine treatment. Second group: patients controlled with L-thyroxine substitutive treatment. Audiometric threshold study comparing between the groups of patients and a group of 209 controls was performed. All patients underwent complete otorhinolaryngological examination, antithyroid antibodies, TSH, T3 and T4 blood levels, tympanometry, conventional pure-tone audiometry, and extended-high-frequency audiometry. RESULTS: All patients were women. Both groups showed worst audiometric thresholds than the control group; both study groups showed worse hearing than controls, this difference was statistically significant in all frequencies. In the 8-20kHz frequency range, this difference was more than 10dB, and in the 9-16kHz and 20kHz range this difference was more than 20dB. When separated by age groups, in younger subjects (20-29 years) these differences were found in all frequencies, except for conversational frequencies (500-4,000Hz); between 30 and 49 years the difference is statistically significant in all frequencies; and from 50 to 69 years differences are found, especially in the conversational frequencies. CONCLUSIONS: This first work studying the human auditory range in the chronic autoimmune thyroiditis or Hashimoto's thyroiditis confirms that hearing loss related to the autoimmune disorder predominates at extended-high-frequencies initially. But ends up involving all frequencies in pure-tone conventional audiometry, then it may be detected in routine clinical tests. These results support the role of extended-high-frequencies audiometry to diagnose subclinical hearing loss in patients affected by Hashimoto's thyroiditis.

Immune-Mediated Inner Ear Disease Associated with Type 1 Autoimmune Hepatitis: A Challenging Coexistence.

Autoimmune hepatitis (AIH) is characterized by elevated serum transaminase, increased immunoglobulin G levels, presence of autoantibodies, and hepatocellular damage. Coexistence with other autoimmune diseases has been reported in almost half of patients with AIH. Here, we report a 60-year-old man who developed rapidly progressive, bilateral, asymmetrical, and asynchronous sensorineural hearing loss that was consistent with immune-mediated inner ear disease (IMIED). This devastating presentation evolved as a late manifestation in the context of a six-month systemic illness that had previously resulted in type 1 AIH. A biochemical remission with normalization of aminotransferases achieved within two months after the initiation of corticosteroids with azathioprine. Further, an acceptable response has also been achieved at the patient regarding the right ear-hearing impairment; though, treatment could not reverse the substantial decrement in hearing capability of the left ear. To our knowledge, this is the first case report of the concurrent development of type 1 AIH and IMIED.

Hearing loss in autoimmune disorders: Prevalence and therapeutic options.

The objective of this study was to review our current knowledge relative to the correlation between sensorineural hearing loss (SNHL) and autoimmune diseases, focusing on the prevalence of hearing loss in different pathologies and possible therapeutic approaches. A review of the literature on hearing loss in different forms of autoimmune disease has been carried out, with emphasis on incidence and prevalence of SNHL. Therapeutic protocols have been assessed including both conservative medical and rehabilitative methods. Cochlear implant outcomes have been investigated. The prevalence of hearing loss in autoimmune and immune-mediated inner ear diseases, as referred by case reports or single-center statistics, is widely variable. More difficult is the evaluation of severe/profound SNHL, usually reported in relation to cochlear implantation. Though these patients represent ideal candidates for cochlear implantation, as they become deaf after years of hearing; the associated systemic disease, the specific damage on inner ear structures and the medication taken may influence the result of cochlear implantation. The main problem is the cochlear fibrosis or ossification that has been found to affect 50% of implanted ears in patients suffering from autoimmune and immune-mediated SNHL. Hence, in the presence of severe/profound SNHL earlier implantation may be indicated before post-inflammatory obliterative changes to the cochlea.

Fluctuating Hearing Loss in the Only Hearing Ear: Cochlear Implantation in the Contralateral Deaf Side.

Objective To investigate the hearing performance of adult patients presenting unilateral deafness with contralateral fluctuating hearing loss who received a cochlear implant on the deaf side. Study Design Case series with chart review. Setting University tertiary referral center. Subjects and Methods Preoperatively and at 6 and 12 months postoperatively, 23 patients underwent pure tone audiometry and speech audiometry with disyllabic and monosyllabic words in a quiet environment and sentences in quiet and noisy (signal-to-noise ratio +10 dB SPL) environments under best-aided conditions. The Abbreviated Profile of Hearing Aid Benefit (APHAB) inventory was evaluated preoperatively and at 6 and 12 months postoperatively. Results No difference was found between pre- and postoperative tests for disyllabic and monosyllabic words. For sentences in quiet and noisy environments, a difference between pre- and postoperative performance was present at 1 year ( P = .002 and P = .02, respectively). In a noisy environment, a difference was present at 6 and 12 months postoperatively as compared with the preoperative value (mean ± SD: 6 months: 42% ± 7.1% vs 61% ± 6.5%, P = .016). A significant improvement in the APHAB score was found at 6 and 12 months postimplantation (Friedman's 2-way analysis of variance by ranks, P < .001). The number of years of hearing deprivation of the deaf ear was not correlated with performance. Conclusion When incapacitating fluctuating hearing loss occurs in patients presenting a contralateral deaf ear, a cochlear implant is indicated in the latter ear, significantly improving performance in noisy conditions and allowing a better quality of communication to be achieved.

Vestibular evoked myogenic potentials in patients with ankylosing spondylitis.

Ankylosing spondylitis (AS) is a chronic systemic inflammatory disease with unknown etiology. Although sacroiliac joint involvement is the classic sign along with the formed immune mediators, it may result in immune-mediated inner ear disease and may cause damage to the audiovestibular system. Vestibular evoked myogenic potentials (VEMP) is a clinical reflex test used in the diagnosis of vestibular diseases and is performed by recording and evaluating the muscle potentials resulting from the stimulation of the vestibular system with different stimuli. The aim of this study is to evaluate the cervical VEMP test results in AS patients without vestibular symptoms. Thirty-three patients with AS and a control group of 30 healthy volunteers with similar demographic characteristics were evaluated in the study. VEMP wave latency, P13-N23 wave amplitude, and VEMP asymmetry ratio (VAR) values were compared between the groups. The relationship between clinical and laboratory findings of the AS patients and VEMP data were also investigated. Compared with healthy people, this study shows the response rate of patients with ankylosing spondylitis was reduced in the VEMP test, and P13-N23 wave amplitude showed a decrease in AS patients who had VEMP response (p < 0.001). There was no correlation between the clinical and laboratory findings and VEMP findings in patients with ankylosing spondylitis. The data obtained from this study suggest that AS may lead to decreased sensitivity of the vestibular system.

Model of immune-mediated inner ear disease and efficacy of dexamethasone by systemic application or round window administration / 上海第二医科大学学报

Objective To establish a model of immune-mediated inner ear disease(IMIED) and to compare the efficacy of dexamethasone between systemic application and round window adminstration. Methods The guinea pigs were injected with keyhole limpet hemocyanin(KLH) mixed with complete Freund's adjuvant subcutaneouly in the back,and were boosted with KLH mixed with incomplete Freund's adjuvant two weeks later.Meanwhile,a small piece of gelatin sponge full of KLH was delivered on the round window through operation.Thus established a model of IMIED.Micro-osmotic pumps were used to deliver dexamethasone through the round window(PBS control group also set),and systemic application was implemented by intraperitoneal injection.The efficacy was compared by recording auditory brain-stem response(ABR) before and after the treatment. Results None of the 6 guinea pigs in the control group experienced significant hearing loss,while 22 of the 39 guinea pigs in the experiment group suffered from hearing loss beyond 10 dB.Seventeen guinea pigs with hearing loss no less than 15 dB were divided into three groups randomly and were given different treatment.Those(n=6) treated by local application through the round window enjoyed the efficacy and the mean ABR threshold decreased 13.3 dB.Four out of the 6 treated by systemic application enjoyed the efficacy and the mean ABR threshold decreased 13.7 dB.No efficacy was observed in the PBS group. Conclusion The model of IMIED can be successfully induce by KLH through the round window,and dexamethasone administered through the round window is as efficacious as systemic application.