An Evaluation of the Relationship Between Professional Self-Esteem and Job Satisfaction Levels of Audiologists According to Their Workplace Types.

BACKGROUND: The concept of occupational self-esteem is the value judgment developed towards the chosen profession, while job satisfaction includes positive emotional attitudes towards the job. Professional self-esteem and job satisfaction levels of audiologists are a matter of curiosity. PURPOSE: To determine the effect of the workplace types of audiologists in Turkey on their professional self-esteem and job satisfaction levels. STUDY SAMPLE: The study included 307 audiologists working in various types of workplace. Participation in the study was voluntary. DATA COLLECTION: A Personal Information Form, Aricak's Occupational Self-Esteem Scale, and the Minnesota Job Satisfaction Scale were administered in all individuals via Google forms and the scale scores were compared according to the workplace types. RESULTS: A statistically significant relationship was found between professional self-esteem and job satisfaction, age, satisfaction with workplace, satisfaction with working conditions, effect of workplace on professional development, and income satisfaction (p<0.05). However, no statistically significant relationship was found between professional self-esteem and professional tenure and tenure in the current organization (p>0.05). There was a strong positive statistically significant relationship between job satisfaction and professional self-esteem of audiologists working at state hospitals, hearing aid centers, university hospitals, rehabilitation centers, and cochlear implant centers and academics (p<0.05). CONCLUSIONS: According to the present results, although the professional self-esteem of audiologists in Turkey showed a small difference according to the type of workplace, their mean score was high. This shows that audiologists enjoy their profession. Job satisfaction levels were lower especially in rehabilitation centers and private hospitals. We think that improving the working conditions of audiologists in private institutions will be effective in improving the services provided to patients by leading to better job satisfaction.

Clinical Interpretation of Positional Nystagmus Provoked by both Dix-Hallpike and Supine Head-Roll Tests.

BACKGROUND: Both the Dix-Hallpike test and the supine head-roll test can provoke positional nystagmus in a group of benign paroxysmal positional vertigo patients, including but not limited to those with multiple canal involvement. This study aimed to determine the incidence and interpret the clinical significance of positional nystagmus provoked by both the Dix-Hallpike and the supine head-roll tests. METHODS: The results of video-nystagmography sessions recorded in the computer database that included both the Dix-Hallpike and the supine head-roll tests were examined. RESULTS: The records belonging to 2880 video-nystagmography sessions of 2387 patients were examined. Nystagmus was detected in both the Dix-Hallpike and the supine head-roll tests of 131 (5.5%) patients. The video images belonging to 142 session records of 122 patients were accessed and further analyzed. The diagnosis was posterior canal BPPV in 9.0%, and lateral canal BPPV in 62.3%. More than one canal was involved in 3.3%, one rehabilitation maneuver was performed in 75.0%, and recurrence was observed in 7.4% of those patients. CONCLUSION: In both geotropic and apogeotropic variants of lateral canal BPPV, nystagmus can be observed during the Dix-Hallpike test in addition to the supine head-roll test. In patients with posterior canal benign paroxysmal positional vertigo, nystagmus can also be observed in the head-roll test. To reach a correct and comprehensive diagnosis and apply appropriate treatment in benign paroxysmal positional vertigo, the Dix-Hallpike test and the head-roll test should be completely performed on both sides, and the results of those tests must be interpreted concomitantly.

Middle-Ear Resonance Frequency and Eustachian Tube Function in Players of Wind Instruments.

INTRODUCTION: The effect of the continuous forced expiration action of players of wind instruments to produce sound, on the eustachian tube functions and the middle-ear resonance frequency (RF), has not been investigated in the literature to date. The aim of this study is to evaluate eustachian tube functions and the middle-ear RF of players of wind instruments. METHODS: In this prospective case-control clinical study, a study group of 28 players of wind instruments in the orchestra (28 participants, 56 ears) and a control group of 34 volunteers (34 participants, 68 ears) were included. The eustachian function of wind instrument players in a symphony orchestra was measured using an automatic eustachian tube function test in acoustic tympanometry and the RF of the middle ear was determined in multifrequency tympanometry. RESULTS: There was a statistically significant difference among the musicians, especially in players of woodwind instruments, in terms of dysfunction of the eustachian tubes (p = 0.048). In the musicians, the pre- and postperformance RF mean values for all ears were 925 and 1,020 Hz, respectively, and these were significantly different (p = 0.004). CONCLUSION: This is the first study to uses multifrequency tympanometry to examine the middle-ear RF and eustachian tube function of wind instrument musicians in an orchestra. Eustachian tube dysfunction was found to be more prominent and a higher RF of the middle ear was seen after a performance, especially in players of wood wind instruments. However, the effect of these on the professional performance of players of wind instruments should be investigated in future work.

Medial olivary complex reflex in term newborns with hyperbilirubinemia.

OBJECTIVE: This study aimed to compare the integrity of the efferent auditory pathways of newborns that had high hyperbilirubinemia levels and required treatment due to these and healthy newborns. METHODS: Term-born (37 weeks or later) infants that were brought to the Newborn Polyclinic of the Baskent University Hospital were included in the study. The study included a total of 84 infants including healthy newborns (n = 42) and those that had jaundice and were receiving phototherapy (n = 42). After conducting a general otorhinolaryngology examination on all newborns included in the study, Transient Otoacoustic Emission (TEOAE) test was carried out in the absence and presence of contralateral noise. The obtained contralateral suppression values were compared between the two groups. RESULTS: In the TEOAE test, the responses obtained at 1 kHz in the newborns receiving phototherapy were found to be lower. The difference between the groups was significant (p = 0.038). The rates of suppression presence at 2 kHz, 2.8 kHz and total OAE were found significantly higher (p < 0.05) in the group not receiving phototherapy. Among the phototherapy-receiving infants, the hyperbilirubinemia levels of the infants in whom suppression was obtained in the contralateral suppression test did not show a statistically significant difference in comparison to those in whom suppression was not obtained (p > 0.05). CONCLUSION: Based on the obtained data, hyperbilirubinemia may have a disruptive effect on the efferent auditory system in newborns. Consequently, we are of the opinion that, in addition to hearing screening in risky newborn infants, a MOC suppression test would be useful.

Evaluation of the possible effect of magnetic resonance imaging noise on peripheral hearing organ with the otoacoustic emission.

PURPOSE: The aim of this study is to evaluate the effect of noise produced by magnetic resonance imaging (MRI) device on hearing by using objective and subjective audiological assessments. METHODS: A total of 38 patients between the ages of 18 and 50 without hearing loss, and had performed MRI for brain, head, neck or cervical imaging were included in this prospective clinical study. Pure tone audiometry, speech audiometry, high frequency audiometry, transient evoked otoacoustic emissions (TEOAE) and distortion product otoacoustic emission (DPOAE) were performed before and after MRI. RESULTS: There was no statistically significant difference in TEOAE, pure tone audiogram, high frequency audiogram and speech audiogram thresholds. In DPOAE, the median value before and after MRI at the frequency of the left ear at 4.0 kHz was 13.6 (8.5-19.9) and 15.7 (8.9-20.7) SNR respectively (p > .05). The median value before MRI at the right ear 4.0 kHz frequency was 14.1 (9.1-20.5) SNR, whereas the median value after MRI was 13.2 (8.8-19.8 SNR (p = 0,03). There was no statistically significant difference in other frequencies in DPOAE. CONCLUSIONS: This is the first objective study that examines the MRI noise on speech audiometry and otoacoustic emission together. However, the effect of MRI noise on hearing pathway is still doubt. Based on the difference at 4 kHz frequency on DPOAE; on-earphones may not sufficiently protect the patients from the MRI noise and this issue should deserve further research.

The protective effect of adrenocorticotropic hormone treatment against noise-induced hearing loss.

OBJECTIVE: NIHL is a common problem, and steroids are the most effective treatment option. In this study, we aimed to evaluate the protective effects of the synthetic adrenocorticotropic hormone (ACTH) analogues, which induce endogenous steroid secretion, against noise-induced hearing loss (NIHL) and to compare their effectiveness with that of steroid treatment. METHODS: Twenty-four male Sprague-Dawley albino rats were divided into four subgroups as follows: group 1 (n=6) control, group 2 (n=6) saline, group 3 (n=6) dexamethasone (2mg/kg/day intramuscularly [IM]), group 4 (n=6) ACTH analogue (0,4mg/kg/day IM), respectively. Three groups (groups 2-4) were exposed to white noise (105dB SPL, 12h). All the rats were evaluated for hearing thresholds of 10kHz, 20kHz, and 32kHz via acoustic brainstem responses (ABR) measurement. After the basal threshold measurements, measurements were repeated immediately after the noise and on day 7 and day 21. RESULTS: Both steroid and ACTH analogue groups showed significantly better hearing outcomes than the saline group on day 7 (p<0.001) and day 21 (p<0.001) after the noise exposure. No superior treatment effect was demonstrated in either the steroid or ACTH analogue group. None of the related intervention groups reached the basal hearing thresholds. CONCLUSION: Steroids were effective drugs for the treatment of NIHL. ACTH analogues also demonstrated promising therapeutic effects for NIHL. Further studies to establish ACTH analogues as an alternative NIHL treatment option to steroids are needed.

Protective Effect of Nigella Sativa Oil on Acoustic Trauma Induced Hearing Loss in Rats.

Acoustic trauma is a common reason for hearing loss. Different agents are used to prevent the harmful effect of acoustic trauma on hearing. The aim of this study was to evaluate the potential preventive effect of Nigella sativa (black cumin) oil in acoustic trauma. Our experimental study was conducted with 20 Sprague Downey female rats (mean age, 12 months; mean weight 250 g). All of the procedures were held under general anesthesia. Following otoscopic examinations, baseline-hearing thresholds were obtained using auditory brainstem responses (ABR). To create acoustic trauma, the rats were then exposed to white band noise of 4 kHz with an intensity level of 107 dB in a soundproof testing room. On Day 1 following acoustic trauma, hearing threshold measurements were repeated. The rats were divided into two groups as the study group (n: 10) and the controls (n: 10). 2 mL/kg/day of Nigella sativa oil was given to the rats in the study group orally. On Day 4 following acoustic trauma, ABR measurements were repeated again. There was no difference between the baseline hearing thresholds of the rats before acoustic trauma (P>0.005). After the acoustic trauma, hearing thresholds were increased and there was no significant statistically difference between the hearing thresholds of the study and control groups (P=0.979). At the 4th day following acoustic trauma, hearing thresholds of the rats in control group were found to be higher than those in the study group (P=0.03). Our results suggest that Nigella sativa oil has a protective effect against acoustic trauma in early period. This finding should be supported with additional experimental and clinical studies, especially to determine the optimal dose, duration and frequency of potential Nigella sativa oil therapy.

Decrease in Middle Ear Resonance Frequency During Pregnancy.

Many physiological changes occur during pregnancy. The aim of the study was to reveal whether there is a change in middle ear resonance frequency during pregnancy. A prospective case-control study was designed at a tertiary referral center. The study included 46 pregnant women at the third trimester (27-40 weeks) and 43 nonpregnant voluntary women. All the study subjects underwent pure-tone audiometry and multifrequency tympanometry. Pure-tone hearing levels at frequencies of 250 to 8000 Hz and resonance frequency values were compared between pregnant and nonpregnant women. Impact of age, side of the tested ear, and weight gained in pregnancy on resonance frequency were evaluated. Air conduction threshold values at frequencies of 250 Hz and 500 Hz were significantly higher in pregnant women than in the control group (P<0.001). Middle ear resonance frequency values of both ears in pregnant women were found to be significantly lower than those in control group (P<0.001). There was no statistically significant relation of middle ear resonance frequency values to age or side of the tested ear in both groups (P>0.05). A negative correlation between weight gained in pregnancy and middle ear resonance frequency values was determined for the left ear (correlation coefficient for left ears: -0.348, P=0.018). The results of this study suggest that resonance frequency may be decreased during the pregnancy. More comprehensive studies in which many pregnant women followed regularly before and after pregnancy are needed to have more certain links.

Vestibular-evoked myogenic potentials before and after dental implant surgery.

OBJECTIVES: This study aims to examine the effects of dental implant surgery on vestibular-evoked myogenic potential (VEMP) responses. PATIENTS AND METHODS: Between October 2012 and October 2014, a total of 60 consecutive patients undergoing dental implant surgery at Baskent University Hospital, Department of Otorhinolaryngology were included in the study. The control group consisted of 40 healthy subjects undergoing routine clinical examination. The VEMP response was evaluated at baseline, second and seventh days. Positional tests and Dix-Hallpike testing for vertigo were performed at baseline, second and seventh days. RESULTS: Hundred-eight dental implants were placed in 60 patients. There was no significant difference in the p1 and n1 latencies in the control group at baseline, second day, and seventh day (p>0.05). However, there was a significant increase at the second day for p1 latencies in the study group, compared to the baseline and seventh day (p=0.038). There was a significant increase at the second day for n1 compared to the baseline (p=0.016) and seventh day in the study group (p=0.005). There was a significant increase at the seventh day for n1 compared to the baseline in the study group (p=0.016). CONCLUSION: The present study revealed a significant difference in the cervical VEMP response on the second postoperative day for p1 and n1 latencies compared to the baseline and seventh postoperative day in dental implantation patients. Dental implant patients should be informed of such possible temporary problems as dizziness and vestibular problems.

Glycerol affects vestibular-evoked myogenic potentials and pure-tone hearing in patients with Ménière's disease.

CONCLUSION: The pure-tone audiometry results following glycerol administration indicated a positive effect on cochlear endolymphatic hydrops. Glycerol cervical vestibular-evoked myogenic potential (cVEMP) tests are a useful means of diagnosing saccular hydrops. There was no correlation between cVEMP and audiological results. OBJECTIVE: To document the changes in pure-tone hearing outcomes and cVEMPs in patients with Ménière's disease (MD) and 10 healthy volunteers before and after oral administration of glycerol. METHODS: Twenty-nine study group subjects were chosen with complaints of vertigo. cVEMP testing and pure-tone hearing level testing were performed before and at 1, 2, and 3 h after administration of glycerol. RESULTS: The means of the latencies, amplitudes, and difference ratio in 20 normal subject ears were determined. Based on these values, 9/29 MD-affected (MDA) ears (31%) had a unilaterally absent cVEMP. Compared with difference ratio values of the control groups there were significant differences in both latencies and amplitudes in MDA ears after glycerol administration. Before glycerol administration, there were significant differences between control and MDA ears on mean values of pure-tone hearing outcomes. Twenty patients in the MDA group showed significant pure-tone hearing outcomes after glycerol administration.

Ocular vestibular evoked myogenic potentials in response to air conducted stimuli: clinical application in healthy adults.

OBJECTIVES: This study aims to determine the normal values for ocular vestibular evoked myogenic potentials in response to air conducted stimuli in healthy adults. PATIENTS AND METHODS: Thirty-six healthy adult participants with no ear complaints were enrolled. Ocular vestibular evoked myogenic potential tests were performed to all participants. Latency and amplitude values of the waves were recorded. RESULTS: The mean N1 latency was 9.62±2.02 (4.30-16.00) msec and the mean P1 latency was 14.90±2.33 (9.0-21.00) msec. The mean amplitude was 3.36±1.36 (1.06-8.48) µV. There was a positive correlation between N1 and P1 latencies and age (r=242, p=0.0359 for N1; r=250, p=0.030 for P1). CONCLUSION: Ocular vestibular evoked myogenic potentials can be obtained easily and can be used in the evaluation of vestibular disorders. However, the effect of age should be considered when interpreting results.

[Normative values of middle ear resonance frequency in normal hearing adults]. / Normal isiten yetiskinlerde orta kulak rezonans frekansi normatif degerleri.

OBJECTIVES: This study aims to determine the normative values of middle ear resonance frequency in healthy adults. PATIENTS AND METHODS: Sixty adult volunteers (32 females, 28 males; mean age 31.8±7.1 years; range 21 to 46 years) with normal otoscopic examination, audiometry and electroacoustic immitancemetry findings were enrolled in the study. The middle ear resonance frequencies were calculated by multifrequency tympanometry. RESULTS: The mean resonance frequency for all volunteers was 999.6±134.9 Hz. The mean resonance frequency was 1020.8±140.6 Hz for the right ear, and 978.3±180.5 Hz for the left ear. The mean resonance frequencies for the right- and left-side were 1023.2±146.9 Hz and 912.5±177.8 Hz in males, and 1018.8±137.2 Hz and 1035.9±164.7 Hz in females, respectively. There was no statistically significant difference in the mean resonance frequency of the right ears between the males and females (p=0.9), whereas the mean resonance frequency of the left ears was statistically significantly higher in females (p=0.007). CONCLUSION: The middle ear resonance frequency values may vary according to the side of the ear or gender.

Effectiveness of the combined hearing and masking devices on the severity and perception of tinnitus: a randomized, controlled, double-blind study.

OBJECTIVE: The aim of this study was to evaluate the effect of combined hearing and tinnitus masking devices that are appropriately programmed for acoustic stimulations using wide-band noise over the specific frequency range of tinnitus. MATERIAL AND METHODS: A total of 21 patients were randomly divided into 2 groups. Group I (12 patients) was managed with betahistine dihydrochloride (2HCl) and fitted either with a combined hearing aid or a sound generator, and group II (9 patients) was treated with betahistine 2HCl for 3 months. Audiological tests, pitch matching to determine the frequency of tinnitus, an assessment of tinnitus severity, and subjective scores (visual analog scale, VAS; Mini-Tinnitus Questionnaire) were used to assess the patients in both groups, and a loudness scale was also analyzed in group I. The results were evaluated in a double-blinded manner. RESULTS: Significant decreases in the severity of tinnitus, Mini-Tinnitus Questionnaire score and VAS were observed in both groups. No significant differences were obtained in pitch-matched frequency of tinnitus in the two groups. CONCLUSION: The findings obtained using either the combined devices or the masking devices with wide-band masking demonstrate that these devices are an effective tinnitus treatment alternative.

Vestibular evoked myogenic potentials in term newborn infants with severe hyperbilirubinemia.

BACKGROUND: The aim of this study was to determine the effects of severe hyperbilirubinemia on vestibular evoked myogenic potentials (VEMP). METHODS: A prospective study was designed. Seventeen term infants who suffered from severe hyperbilirubinemia in the first 5 postnatal days of age were included in the study group. The control group consisted of 17 healthy term infants. Audiological evaluation was performed, including tympanometry and transient evoked otoacoustic emissions, and VEMP tests. RESULTS: All newborns passed audiological evaluation. Biphasic waveforms of VEMP were obtained in all of the 34 infants who had been tested. Both latencies of p13 and n23 were significantly delayed in the severe hyperbilirubinemia group (P < 0.05). CONCLUSION: This pilot is the first study to show that severe hyperbilirubinemia causes delay in VEMP latencies. We suggest that severe hyperbilirubinemia might affect the vestibular nuclei or the integrity of the inferior vestibular nerve and vestibulospinal tract. Further studies need to explain the relation between hyperbilirubinemia and the vestibular system.

Comparison of cervical vestibular evoked myogenic potentials between late preterm and term infants.

Recent investigations have shown that late preterm infants have increased risk for attention deficit hyperactivity disorder, neurosensory impairment, and emotional, behavior and learning problems. Vestibular evoked myogenic potential (VEMP) abnormality may partly contribute to these problems. Our aim was to measure VEMP in late preterm infants and to compare the findings between late preterm and term infants. Seventeen late preterm infants (mean gestational age: 35.11 weeks +/- 0.78) postnatal aged 8 weeks and 17 full-term (mean gestational age: 38.05 weeks +/- 0.96) infants postnatal aged 4 weeks underwent cervical (c)VEMP test without sedation. Mean latencies of p13 were calculated in all study subjects. cVEMPs were elicited in all late preterm and term infants. Mean latencies of p13 in late preterm and term infants were 14.53 and 13.34 ms, respectively. Mean latencies of n23 were determined as 23.18 ms and 19.92 ms for late preterm and term infants, respectively. There were statistically significant differences between late preterm and term infants for latency of p13 (p < 0.001) and latency of n23 (p < 0.000). Abnormal VEMP results might be related to a delay in the maturation of the sacculocollic pathways in late preterm infants.

Risk of nasal septal perforation following septoplasty in patients with allergic rhinitis.

Nasal septal perforation (NSP) is an uncomfortable condition that usually has a negative effect on the quality of life and is frequently seen as a complication of nasal surgical procedures. The aim of this study was to evaluate the effect of allergic rhinitis (AR) on the occurence rates of septal perforation as a complication of septoplasty. The medical records of 352 patients who had septoplasty at our department between January 2006 and May 2009 were searched thoroughly and those with a follow-up period of at least 1 month were included in the study. Septal perforation rates were compared between two groups, one of which was formed by patients known to have AR besides septum deviation and the other with no history of AR. Of the 352 patients, 70 (19.8%) had accompanying AR. Only three patients (0.9%), two in the non-allergic group (NAG) and one in the allergic group (AG), were noted to have NSP after septoplasty. According to this clinical data, NSP rates were 1.4 and 0.7%, respectively, in AG and NAG. No statistical significance was found between the two groups (p = 0.487). AR had no impact on the risk of NSP after septoplasty. To our opinion, an appropriate surgical technique and a respectful approach to the tissue planes are the key points of avoiding undesired results.

Recurrent and private MYO15A mutations are associated with deafness in the Turkish population.

The identities and frequencies of MYO15A mutations associated with hearing loss in different populations remained largely unknown. We screened the MYO15A gene for mutations in 104 unrelated multiplex and consanguineous Turkish families with autosomal recessive nonsyndromic sensorineural hearing loss using autozygosity mapping. The screening of MYO15A in 10 families mapped to the DFNB3 locus revealed five previously unreported mutations: p.Y289X (1 family), p.V1400M (1 family), p.S1481P (1 family), p.R1937TfsX10 (3 families), and p.S3335AfsX121 (2 families). Recurrent mutations were associated with conserved haplotypes suggesting the presence of founder effects. Severe to profound sensorineural hearing loss was observed in all subjects with homozygous mutations except for two members of a family who were homozygous for the p.Y289X mutation in the N-terminal extension domain and had considerable residual hearing. We estimate the prevalence of homozygous MYO15A mutations in autosomal recessive nonsyndromic deafness in Turkey as 0.062 (95% confidence interval is 0.020-0.105).

Expression of a disintegrin and metalloproteinase 33 protein in nasal polyposis: an immunohistochemical study.

BACKGROUND: A disintegrin and metalloproteinase (ADAM)-33 is a member of matrix metalloproteinases. This protein takes a role in angiogenesis and airway remodeling in asthma. Because histopathological findings of airway remodeling in asthma and nasal polyposis (NP) are similar, the aim of this study was to evaluate the ADAM-33 expression in NP. METHODS: Immunohistochemical staining of specimens of 47 patients with NP and 8 patients with concha bullosa was performed to detect the expression of ADAM-33. Paraffin blocks were used to identify the expression of ADAM-33 polyclonal antibodies. Immunostaining of epithelial cells, stroma, mesenchymal cells of vessels, and inflammatory cells were analyzed by using light microscopy. RESULTS: Immunopositivity scores in epithelial cells in NP (median, 2; range, 1-3) were significantly higher than those of controls (median, 1.5; range, 1-2; p < 0.001). ADAM-33 staining was increased in the mesenchymal cells of vessels of nasal polyps (median, 2; range, 1-3) compared with control tissues (median, 1.5; range, 1-2; p = 0.006). Although the staining scores of fibroblasts in nasal polyp specimens were also high (median, 3; range 1-3), there was no statistical significance when compared with controls (median 2; range, 1-3; p = 0.228). ADAM-33 immunostaining was not related with the presence of allergies, asthma, and aspirin intolerance (p > 0.05). Moreover, no relationship was found between increased expression of ADAM-33 and the stages of polyp or computerized tomography scores (p > 0.05). CONCLUSION: This study suggests that the increased expression of ADAM-33 protein may have a role in the pathogenesis of NP.

A truncating mutation in SERPINB6 is associated with autosomal-recessive nonsyndromic sensorineural hearing loss.

More than 270 million people worldwide have hearing loss that affects normal communication. Although astonishing progress has been made in the identification of more than 50 genes for deafness during the past decade, the majority of deafness genes are yet to be identified. In this study, we mapped a previously unknown autosomal-recessive nonsyndromic sensorineural hearing loss locus (DFNB91) to chromosome 6p25 in a consanguineous Turkish family. The degree of hearing loss was moderate to severe in affected individuals. We subsequently identified a nonsense mutation (p.E245X) in SERPINB6, which is located within the linkage interval for DFNB91 and encodes for an intracellular protease inhibitor. The p.E245X mutation cosegregated in the family as a completely penetrant autosomal-recessive trait and was absent in 300 Turkish controls. The mRNA expression of SERPINB6 was reduced and production of protein was absent in the peripheral leukocytes of homozygotes, suggesting that the hearing loss is due to loss of function of SERPINB6. We also demonstrated that SERPINB6 was expressed primarily in the inner ear hair cells. We propose that SERPINB6 plays an important role in the inner ear in the protection against leakage of lysosomal content during stress and that loss of this protection results in cell death and sensorineural hearing loss.