Progressive Bilateral Sensorineural Hearing Loss Research Articles

Bilateral vestibulopathy and sensorineural hearing loss in necrobiotic xanthogranuloma, multiple myeloma and amiloidosis

This article describes a rare case of necrotic xanthogranuloma in a 46-year-old patient who presented with the development of periorbital xanthelasms, progressive bilateral sensorineural hearing loss and bilateral vestibulopathy, followed by multiple myeloma and amyloidosis. For several years, the patient underwent standard rehabilitation for chronic sensorineural hearing loss and was fitted with a hearing aid. During hospitalisation for exacerbation of chronic bronchitis, monoclonal gammopathy was identified, and later, after careful examination and repeated biopsies, necrotic xanthogranuloma, multiple myeloma and AL-amyloidosis were confirmed. Targeted immunochemotherapy resulted in improvement of hearing and significant recovery of the vestibuloocular reflex bilaterally.

Hyperglycemia with hypogonadism and growth hormone deficiency in a 17-year-old male with H syndrome: the first case report from Syria

BackgroundThe nucleoside transport capabilities of the human equilibrative nucleoside transporter-3 (hENT3) are disrupted by mutations in SLC29A3 (10q22.2), which are genes for the nucleoside transporter and are the cause of the unusual autosomal recessive disease known as H syndrome. As a result, histiocytic cells invade a number of organs.Case presentationA 17-year-old Syrian male was admitted to the internal medicine department with a one-month history of polyuria, polydipsia, general weakness, and pallor. He had a history of progressive bilateral sensorineural hearing loss and failure to gain weight for three years. Physical examination revealed various abnormalities, including scrotal mass, small penis and testicles, absence of pubic and axillary hair, joint abnormalities, short stature, hallux valgus, fibrous protrusion near the navel, and hyperpigmented non-itchy painful skin plaques. Clinical signs along with laboratory test results confirmed hyperglycemia, primary hypogonadism, osteopenia, and growth hormone deficiency. After a review of the relevant medical literature, this patient’s presentation of hyperglycemia with hypogonadism, hyperpigmentation, hallux valgus, hearing loss, hematological abnormalities, and short stature suggested the diagnosis of H syndrome. The patient received treatment with insulin and testosterone, leading to a significant improvement in his presenting symptoms.ConclusionsH syndrome is a very rare condition, and the fact that the first case has only recently been reported in Syria serves to emphasize how rare it is. H Syndrome should be suspected if a patient has short stature with signs of hyperglycemia and other endocrine and cutaneous abnormalities. We are reporting this case to increase physicians’ awareness of this exceedingly rare and unique syndrome.

A Retrospective Study of Clinical Profile of Patients with Autoimmune Inner Ear Disease.

Bilateral sensorineural hearing loss can be a very distressing symptom and can affect the efficiency of a person and one's quality of life. Conditions causing bilateral hearing loss are very few and autoimmune aetiology is one of them. Autoimmune ear disease is characterised by bilateral, mostly fluctuating audiovestibular symptoms and symptoms which respond to steroids. Diagnosis of AIED presents a unique challenge to clinicians due to the lack of standardized diagnostic criteria or reliable pathognomonic tests. The purpose of the study is to evaluate the patients who fit into criteria of autoimmune inner ear disease and understand the clinical features and response to medications for the same. A retrospective chart review of patients presenting with rapidly progressive bilateral hearing loss was done. The clinical presentation including detailed history and examination findings along with the blood investigation reports and audiograms were recorded in a tabular form. The study included 6 patients - 3 male and 3 female patients. Age of the patients at onset of hearing loss varied between 24-35 years. 3 of 6 patients presented with primary autoimmune ear disease and other 3 had hearing loss secondary to systemic autoimmune disease. All patients were treated with systemic steroids, but however showed a varied response. Patients with primary AIED were administered inner ear steroid therapy as well. AIED is thus a diagnosis of exclusion done with high index of suspicion. Patients with bilateral progressive sensorineural hearing loss should be evaluated for autoimmune etiology. Oral steroids with intratympanic steroids are currently the mainstay of treatment for AIED. Guarded prognosis of hearing improvement is noted in these patients. Hence, emphasis should be placed on early hearing rehabilitation for better quality of life.

Bilateral Sensorineural Hearing Loss Related to a Skull Base Spontaneous Cerebrospinal Fluid Leak: A Case Report and Literature Review.

Spontaneous intracranial hypotension (SIH) is a rare condition characterized by orthostatic headache, pulsatile tinnitus, vertigo, nausea, and fluctuating hearing loss; this latter seems to be due to the development of cochlear endolymphatic hydrops following negative cerebrospinal fluid (CSF) pressure transmitted to the perilymphatic space through a patent cochlear aqueduct. We here describe a case of bilateral progressive sensorineural hearing loss (SNHL) due to intracranial hypotension caused by an undiagnosed spontaneous CSF leak from a skull base defect in a middle-aged woman. To the best of our knowledge, this is the first report describing a SNHL in a patient affected by SIH secondary to sphenoidal CSF leak.

A Woman With Kelch-like Protein-11 Encephalitis and Unmasked Metastatic Carcinoma

Objective To illustrate a case of a woman with rhombencephalitis with antibodies to Kelch-like protein-11 (KLHL11) and a metastatic carcinoma. Background KLHL11 encephalitis is an autoimmune paraneoplastic syndrome first described in 2019. The clinical presentation consists of a brainstem cerebellar syndrome with symptoms of hearing loss, diplopia, vertigo and ataxia. This entity has been mostly described in male patients with associated testicular seminomas. Few cases have been described in women. This is a case of a woman with a history of hysterectomy and oophorectomy with KLHL11 encephalitis and an associated aortocaval tumor. Design/Methods NA. Results The patient was a 62-year-old woman that presented to clinic with a 9-month history of vertigo, progressive bilateral sensorineural hearing loss, diplopia, oscillopsia, ataxia and bilateral tremor. Her MRI brain obtained 8 months after symptom onset showed T2 hyperintense lesions in the inferior cerebellar hemispheres and right medial hippocampus with mild contrast enhancement in these areas. Cerebral spinal fluid analysis showed a lymphocytic pleocytosis, elevated protein, and negative infectious work-up. She completed 5 days of intravenous methylprednisolone and continued a steroid taper. She noted mild to moderate improvement in tremors, gait, and diplopia after steroids. Her symptoms, however worsened as she tapered her steroid dose. Serum KLHL11 antibody levels were positive at a titer of 1:7680. Computed tomography of the chest, abdomen and pelvis did not reveal any evidence of malignancy. However, whole body proton emission tomography/computed tomography (PET CT) revealed a large hypermetabolic aortocaval mass soft tissue mass. A biopsy of the mass showed pathology consistent with a metastatic carcinoma of gynecologic origin for which the patient is undergoing chemotherapy with plans for possible tumor debulking. Conclusions This case highlights the importance of considering KLHL-11 encephalitis in female patients presenting with rhombencephalitis, and the need for adequate malignancy evaluation in this disorder.

Progressive loss of hearing and balance in superficial siderosis due to occult spinal dural defects

PurposeSuperficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension.MethodsRetrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur.ResultsThe diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve.ConclusionsIn patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.

Auditory and Vestibular Characteristics of NLRP3 Inflammasome Related Autoinflammatory Disorders: Monogenic Hearing Loss Can Be Improved by Anti-interleukin-1 Therapy.

Inflammasomes are large multimeric protein complexes which regulate the activation of the proinflammatory cytokines interleukins-1β and−18 and inflammatory cell death called pyroptosis. NLRP1, NLRP3, NLRC4, AIM2, and pyrin can induce the formation of inflammasomes. Of these, the NLRP3 inflammasome is the most well-characterized. Recent studies revealed that variants of the NLRP3 gene cause genetic diseases, including systemic inflammatory syndrome called cryopyrin-associated periodic syndrome (CAPS) and non-syndromic sensorineural hearing loss DFNA34. NLRP3 variants cause CAPS and DFNA34 by constitutively activating the NLRP3 inflammasome and increasing IL-1β release. Patients with CAPS show systemic inflammatory symptoms, and hearing loss is a characteristic feature. Patients with CAPS and DFNA34 show progressive bilateral sensorineural hearing loss. Hearing loss has unique characteristics that can be improved or stabilized by anti-interluekin-1 therapy, although it is usually difficult to alleviate genetic hearing loss by drugs. However, it should be noted that there is a window of opportunity to respond to treatment, and younger patients are most likely to respond. It is important to know the characteristics of CAPS and DFNA34 for early diagnosis, and mutation analysis of NLRP3 will lead to a definite diagnosis. In this review, we summarize the current understanding of the mechanisms of the NLRP3 inflammasome and characteristics of patients with CAPS and DFNA34, especially focused on auditory and vestibular findings.

A newly identified mutation (c.2029 C\u2009>\u2009T) in SLC26A4 gene is associated with enlarged vestibular aqueducts in a Chinese family

BackgroundThe enlarged vestibular aqueduct (EVA), associated with mutations in the SLC26A4 gene, characterized by non-syndromic hearing loss, is an autosomal recessive disorder. Here, we intended to investigate genetic causes of hearing loss in a Han Chinese man.MethodFirst, whole-exome sequencing was performed to identify the gene mutations responsible for hearing loss in the proband. Sanger sequencing was used to verify the candidate mutations detected in the family. Next, we collected blood samples and clinical data from the three-generation pedigree. Finally, SLC26A4 mRNA and protein expression levels were detected by qPCR and western blotting.ResultThe proband suffered from bilateral progressive sensorineural hearing loss with EVA. The sequence analysis of SLC26A4 revealed that the proband and his sister both harbored a compound heterozygous mutation of c.2168A > G/c.2029C > T, inherited from their father and mother respectively. c.2029C > T mutation has not been recorded in the relevant literature previously. Relative mRNA levels of the SLC26A4 gene in individuals carrying a compound heterozygous mutation were significantly lower compared to a heterozygous mutation. SLC26A4 protein levels of 293t cells which transfected with recombinant plasmids [GV219-SLC26A4-mut (c.2029C > T) and GV219-SLC26A4-mut (c.2168A > G/c.2029C > T)] were significantly lower than normal control recombinant plasmids (GV219-SLC26A4-wt).ConclusionThis study found a novel heterozygous mutation c.2029 (exon17) C > T compound with c.2168 (exon19) A > G in the SLC26A4 gene in a patient with EVA. The c.2029 (exon17) C > T mutation is proved to be pathogenic. This finding broadens the spectrum of variants in SLC26A4 gene.

Sensorineural Hearing Loss of Suspected Autoimmune Etiology: Two Cases of Cogan’s Syndrome

Cogan’s syndrome is a rare inflammatory disease characterized by non-syphilitic keratitis and vestibulo-auditory symptoms including hearing loss, tinnitus, and vertigo. Although its precise pathogenesis is not known, Cogan’s syndrome is generally considered an autoimmune disease. This hypothesis is supported by the frequently successful remission of hearing loss after steroid administration and the association with other autoimmune disorders such as rheumatoid arthritis. Medical treatment of Cogan’s syndrome depends on disease severity and on how extensive the disease is. The involvement of inner ear pathology requires systemic corticosteroid therapy. In cases of treatment failure or the need for a corticosteroid-sparing effect, other immunosuppressive drugs can be used. We experienced two patients with typical Cogan’s syndrome, presenting bilateral progressive sensorineural hearing loss and dizziness with ocular involvement, which we have successfully treated with systemic steroid administration and immunosuppressive therapy.

Auditory Neuropathy as the Initial Phenotype for Patients With ATP1A3 c.2452 G > A: Genotype–Phenotype Study and CI Management

Objective: The objective of this study is to analyze the genotype–phenotype correlation of patients with auditory neuropathy (AN), which is a clinical condition featuring normal cochlear responses and abnormal neural responses, and ATP1A3 c.2452 G > A (p.E818K), which has been generally recognized as a genetic cause of cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) syndrome.Methods: Four patients diagnosed as AN by clinical evaluation and otoacoustic emission and auditory brainstem responses were recruited and analyzed by next-generation sequencing to identify candidate disease-causing variants. Sanger sequencing was performed on the patients and their parents to verify the results, and short tandem repeat-based testing was conducted to confirm the biological relationship between the parents and the patients. Furthermore, cochlear implantation (CI) was performed in one AN patient to reconstruct hearing.Results: Four subjects with AN were identified to share a de novo variant, p.E818K in the ATP1A3 gene. Except for the AN phenotype, patients 1 and 2 exhibited varying degrees of neurological symptoms, implying that they can be diagnosed as CAPOS syndrome. During the 15 years follow-up of patient 1, we observed delayed neurological events and progressive bilateral sensorineural hearing loss in pure tone threshold (pure tone audiometry, PTA). Patient 2 underwent CI on his left ear, and the result was poor. The other two patients (patient 3 and patient 4, who were 8 and 6 years old, respectively) denied any neurological symptoms.Conclusion: ATP1A3 p.E818K has rarely been documented in the Chinese AN population. Our study confirms that p.E818K in the ATP1A3 gene is a multiethnic cause of AN in Chinese individuals. Our study further demonstrates the significance of genetic testing for this specific mutation for identifying the special subtype of AN with somewhat favorable CI outcome and offers a more accurate genetic counseling about the specific de novo mutation.

Generation and Genetic Correction of USH2A c.2299delG Mutation in Patient-Derived Induced Pluripotent Stem Cells.

Usher syndrome (USH) is the leading cause of inherited combined hearing and vision loss. As an autosomal recessive trait, it affects 15,000 people in the United States alone and is responsible for ~21% of inherited blindness and 3 to 6% of early childhood deafness. Approximately 2/3 of the patients with Usher syndrome suffer from USH2, of whom 85% have mutations in the USH2A gene. Patients affected by USH2 suffer from congenital bilateral progressive sensorineural hearing loss and retinitis pigmentosa which leads to progressive loss of vision. To study the molecular mechanisms of this disease and develop a gene therapy strategy, we generated human induced pluripotent stem cells (iPSCs) from peripheral blood mononuclear cells (PBMCs) obtained from a patient carrying compound heterozygous variants of USH2A c.2299delG and c.1256G>T and the patient’s healthy sibling. The pluripotency and stability were confirmed by pluripotency cell specific marker expression and molecular karyotyping. Subsequent CRISPR/Cas9 genome editing using a homology repair template was used to successfully correct the USH2A c.2299delG mutation back to normal c.2299G in the generated patient iPSCs to create an isogenic pair of lines. Importantly, this manuscript describes the first use of the recombinant Cas9 and synthetic gRNA ribonucleoprotein complex approach to correct the USH2A c.2299delG without additional genetic effects in patient-derived iPSCs, an approach that is amenable for therapeutic genome editing. This work lays a solid foundation for future ex vivo and in vivo gene therapy investigations and these patient’s iPSCs also provide an unlimited resource for disease modeling and mechanistic studies.

A case of paraneoplastic neurological syndrome with dizziness and bilateral progressive sensorineural hearing loss

A case of paraneoplastic neurological syndrome with dizziness and bilateral progressive sensorineural hearing loss

Utility and value of pre-operative CT and MRI for cochlear implantation in the elderly

PurposeTo determine the utility and value of pre-operative imaging among the elderly population ≥70 y.o. with bilateral progressive sensorineural hearing loss undergoing cochlear implantation. Materials and methodsA retrospective, cross-sectional review was performed at a tertiary referral center between 2010 and 2018 including patients ≥70 y.o. with bilateral presbycusis who underwent preoperative imaging and cochlear implantation. Primary outcome was whether pre-operative imaging changed the surgeon's surgical plan such as side of implant or abort procedure entirely. Patient characteristics including age, sex, side of implant, imaging modality, whether imaging changed surgical plan, and surgical complications were reviewed. One-way analysis of variance with post-hoc tests using the Bonferroni and Fisher's exact test were used to examine differences between groups. Secondary outcome was cost of preoperative imaging. ResultsOne hundred thirty-three patients (mean age 79.38 [5.51 SD]) who underwent a total of 142 surgical cases and 147 total scans. There were 92, 27, and 14 patients who underwent CT, MRI, or both, respectfully (n=133). Of the 142 implants that were placed, preoperative imaging did not reveal a contraindication to placing implant on one side over another. Total cost of imaging was $29,694. Estimated cost if 20% of cochlear implant eligible patients ≥70 y.o. underwent imaging is $7,763,490. ConclusionDecreasing unnecessary preoperative imaging can potentially decrease cost in cochlear implantation. In this sample, preoperative imaging did not affect the surgeon's choice of which side to operate on. However, imaging may provide an anatomic roadmap and contribute to either surgical confidence or caution. With the increasing amount of cochlear implant eligible elderly adults, preoperative imaging needs to be more clearly defined in this unique population.

Labyrinthitis and Labyrinthitis Ossificans - A case report and review of the literature.

Labyrinthitis most commonly results from an infectious and less commonly from an inflammatory process of the inner ear, but it can be associated with temporal bone trauma, hemorrhage, or tumor. This inflammation (regardless of the etiology) disrupts the transmission of sensory information from the ear to the brain. Labyrinthitis ossificans is the pathological ossification of the membranous labyrinthine spaces in response to an insult to the inner ear involving membranous labyrinth or the endosteum of the otic capsule. Herein, we present a case of a 67-year-old female with progressive bilateral sensorineural hearing loss and positional vertigo.

Cochlear implantation in patient with Charcot-Marie-Tooth disease

Two patients with auditory neuropathy spectrum disorder (ANSD) considered to be associated with Charcot-Marie-Tooth (CMT) are reported. In case 1, a 23-year-old man presented with progressive bilateral sensorineural hearing loss (SNHL) from 10 years of age and was diagnosed with ANSD. He was later diagnosed with CMT by neurological testing. In case 2, a 16-year-old girl, the younger sister of the patient in case 1, presented with progressive SNHL with similar auditory findings since 6 years of age as those of her brother. Both cases underwent bilateral cochlear implantation. In case 1, the maximum discrimination score improved to 45% 24 months after the first side of cochlear implantation from 5% before the surgery. In case 2, the score was 5% 10 months after cochlear implant (CI) surgery from 0% before the surgery. CI treatment for CMT patients has been considered difficult because of both failure in synchronization of nerve conduction due to demyelination and axonal failure of the auditory nerve. Though slower progress compared to the average subset of patients receiving CI was seen, significant improvement was gradually observed in the present patients after bilateral cochlear implantations. CI is thus a viable option for rehabilitation of SNHL in CMT patients.

A Case of Lung Adenocarcinoma with Bilateral Progressive Sensorineural Hearing Loss due to Meningeal Carcinomatosis

A Case of Lung Adenocarcinoma with Bilateral Progressive Sensorineural Hearing Loss due to Meningeal Carcinomatosis

Audiovestibular Phenotypes and Advanced Magnetic Resonance Imaging Features of Cochlin Gene Mutation Carriers

Objective: To describe clinical and imaging findings in a group of patients affected by nonsyndromic deafness A9 (DFNA9), using advanced magnetic resonance imaging (MRI) with 3-dimensional (3D) fluid-attenuated inversion recovery (FLAIR) sequence. Method: A retrospective case review was conducted in a tertiary referral center in Italy. Four sequential adult DFNA9-affected patients, who had undergone MRI at our Department between January 2017 and June 2018, were enrolled (male = 2, female = 2; median age: 65.6 years; 8 diseased ears analyzed). Three patients were relatives; the fourth was unrelated. The main outcome measures – age, sex, records of audiological and vestibular testing, genetic assessment, MRI findings – were analyzed. Results: All subjects suffered from bilateral progressive sensorineural hearing loss, more severely at the high frequencies and with a typical clinical pattern of bilateral chronic degenerative cochleovestibular deficit. Aural fullness was reported at the onset of the disease. All patients revealed a pathogenic heterozygous mutation in the Limulus factor C, Coch-5b2 and Lgl1 domain of cochlin. None of the patients showed a significant vestibular and cochlear endolymphatic hydrops at MRI, while high bilateral contrast enhancement on 4-h delayed postcontrast 3D FLAIR sequence was observed in all ears. Conclusions: Increased perilymph enhancement on 4-h delayed postcontrast 3D FLAIR sequence is the common imaging feature of DFNA9 ears, suggesting that blood-labyrinthine barrier breakdown may play the main role in the pathophysiology of this disease. Significant hydrops has been excluded by MRI. This finding might be clinically useful in differentiating DFNA9 disease from other pathologies with similar clinical findings like Ménière’s disease.

Comparison of Otoacoustic Emissions in Asymptomatic Diabetes Mellitus and Normal Hearing Control Groups

Background: Diabetes mellitus (DM) is a chronic metabolic disorder. Previous studies reported the hearing impairment of diabetes patients as a progressive bilateral symmetrical sensorineural hearing loss (SNHL) of gradual onset which predominantly affected the higher frequencies, and found that diabetic patients had unsatisfactory hearing thresholds than nondiabetics in the same age group. For the use of evoked otoacoustic emissions (EOAEs) as a clinical test of hearing, EOAEs are sound which can be recorded in the outer ear canal and give objective information about preneural, mechanical elements of the cochlear function.
 Objective: To compare the otoacoustic emissions of asymptomatic DM patients with a normal hearing control group.
 Methods: All subjects received a routine audiologic evaluation which consisted of pure-tone air-bone conduction tests, tympanograms, and stapedius acoustic reflex tests. OAEs were measured in 30 asymptomatic diabetic patients with a mean age of 55.73 years and an age-matched 30 subjects normal hearing control group with a mean age of 50.87 years.
 Results: The pure-tone thresholds at 3, 4, 6, and 8 kHz; transient-evoked otoacoustic emissions (TEOAEs) amplitudes at 1, 1.5, 2, and 3 kHz; and the distortion product otoacoustic emissions (DPOAEs) amplitudes at 1, 1.5, 2, 3, 4, and 6 kHz were significantly different (P < .05) between the asymptomatic and control groups.
 Conclusions: This study suggested that diabetic patients had a significant hearing impairment. SNHL is gradually progressive and is a common condition in DM patients and their thresholds for hearing are higher at higher frequencies.

Application of Cochlear Implant for Two Cases of Maternally Inherited Diabetes and Deafness

Maternally inherited diabetes and deafness (MIDD) is a rare subtype form of diabetes caused by a mitochondrial DNA defect associated with m.3243A>G mutation. More than 90% of MIDD cases suffer from bilateral progressive sensorineural hearing loss. Here, we report two cases of MIDD with bilateral profound hearing loss that were successfully treated with cochlear implantation. Case 1 was a 61-year-old man who complained of bilateral progressive hearing loss since the age of 40 years old and who visited our department seeking treatment. He was diagnosed as having MIDD based on a genetic analysis of mitochondrial DNA that revealed a 3243 A to G substitution. Cochlear implantation (CI) was performed for the right ear, which exhibited profound hearing loss. At 5 years after the CI, the patient had acquired hearing function enabling more than 90% of spoken words to be discriminated. Case 2 was a 44-year-old woman who had suffered from diabetes and deafness since the age of 24 years and who had a 3243 A to G substitution in her mitochondrial DNA. She visited our department for cochlear implantation because hearing aids had never improved her hearing. At 6 years after the CI, she had acquired good speech discrimination. We suggest that CI might be a useful treatment for profound hearing loss in patients with MIDD.

Assessment of hearing loss as a potential complication of diabetes mellitus

Introduction: High blood glucose levels are found to damage the vessels found in stria vascularis and adjacent nerves, this results into diminished ability to hear. To support this result, insufficient evidence of a significant relationship between diabetes and hearing impairment is there. Therefore, we aimed to assess hearing impairment in diabetic mellitus individuals as one of the potential complications. Material & Methods: The study involved 150 type 2 Diabetes Mellitus patients. Diabetes Mellitus type 2 subjects 65-89 years of age, meeting the inclusion/exclusion criteria were included in the present study. The individuals with the diabetes had been followed up for 10 years. Results: 89 patients of total 150 patients had duration of diabetes for more than 10 years. Among them mild hearing loss was found in 37 patients. Out of total 150 patients 61 patients in whom the duration of diabetes was less than 10 years, we could not find any detectable hearing loss. Duration of Diabetes mellitus and sensorineural hearing loss at 2000Hz, 4000Hz showed statistically significant correlation (Pearson coefficient r= 0.981 and r = 0.90 respectively) at 0.03 level. Discussion & Conclusion: on the basis of results we came to conclusion that progressive bilateral high frequency sensorineural hearing loss can be considered as one of complication of type 2 DM. Periodical assessment of various organs of the body, including the hearing system, should be carried out carefully and regularly, so as to provide better life condition for the diabetic patients in the future. Keywords: Complications, Diabetis Mellitus, Hearing problem, Presbycusis