X-LINKED MIXED HEARING LOSS

Introduction: Epidermolysis bullosa (EB) is a spectrum of mechanobullous disorders characterized by blistering following minor trauma or traction to the skin. Hearing loss in this population is poorly described in the otolaryngology literature, and its treatment oftentimes results in external auditory canal skin irritation. Case Presentation: We present the case of a 26-year-old female with EB and mixed hearing loss unable to wear conventional hearing aids due to sequelae of the external auditory canals. An osseointegrated implant was used as other hearing aids were deemed to be too destructive of the external auditory canal skin. Management and Outcome: Our patient underwent placement of a right bone-anchored hearing aid with minimal disruption of the surrounding skin using a minimally invasive punch technique. Over 1 year of follow-up, her course was complicated by 1 simple cellulitic infection at the surgical site treated successfully with oral antibiotics. Discussion: The literature regarding the otolaryngologic manifestations of EB is sparse. The otologic sequelae are particularly overlooked in the workup and management. Based on the results of this case study, it appears that an osseointegrated implant can be safely utilized to treat significant mixed or conductive hearing loss in patients with EB.

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Otosclerosis Revisited

Bengal Journal of Otolaryngology and Head Neck Surgery ◽

10.47210/bjohns.2019.v27i1.231 ◽

2019 ◽

Vol 27 (1) ◽

Author(s):

C V Srinivas

Keyword(s):

Hearing Loss ◽

Current Treatment ◽

P Value ◽

College Hospital ◽

Severe Hearing Loss ◽

Medical College ◽

Mixed Hearing Loss ◽

Operative Outcome ◽

Conductive Component ◽

Indian Scenario

Introduction: Otosclerosis usually manifests as a progressive conductive or mixed hearing loss occurring clinically to varying degrees in 0.5% - 1% of the general population. Stapedectomy /stapedotomy is the current treatment of choice for conductive component of Otosclerosis. Materials and Methods: Sixty patients attending the department of ENT of a Medical College Hospital in Bangalore between October 2012 and October 2017 were included in the study. Results: The incidence of otosclerosis is highest in the 3rd and 4th decade. Bilateral symptoms were present in 25% and 75% had unilateral symptoms, right side being more common in our study. Tinnitus and vertigo with deafness are seen in 60% and 25 % of the patients respectively. Ninety percent of the patients presented with moderate to moderately severe hearing loss. Twenty four (40%) out of 60 audiograms studied showed Carhart's notch. Postoperativeclosure of a-b gap to within 10dB could be achieved in 65% cases. The p value calculated comparing pre and post op results is <0.0001 and significant. Vertigo was found to be most common post-operative complication (35%) followed by sensorineural hearing loss (6.6%). Other complications observed during the study include - tympanic membrane tears, foot plate extrusion, lax long process of incus, perilymph leak. Conclusion: From the assessment of the postoperative hearing gain and postoperative complications, it can be concluded that small fenestra stapedotomy gives satisfactory post-operative outcome and may be appropriatein the Indian scenario.

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Assess the hearing of contralateral ear in patients with unilateral chronic squamosal otitis media

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20201680 ◽

2020 ◽

Vol 6 (5) ◽

pp. 886

Author(s):

Raphella Khan ◽

Anirudh Kasliwal

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Otitis Media ◽

Conductive Hearing Loss ◽

Sensorineural Hearing ◽

Retraction Pocket ◽

Mixed Hearing Loss ◽

Contralateral Ear ◽

The Right ◽

Conductive Hearing

Background: Chronic squamosal otitis media can occur due to many conditions affecting the middle ear. Most common sign of developing a chronic squamosal otitis media is formation of a retraction pocket in the tympanic membrane leading to further development of a cholesteatoma and if not treated properly, may lead to development of dangerous complication in the affected ear. These etiological factors may also affect the other ear. It is therefore very necessary to assess and diagnose the contralateral ear, so that the disease can be intervened and treated at the right time, to prevent any deterioration in hearing of the contralateral ear.Methods: The prospective study was done in 100 patients with unilateral chronic squamosal otitis media, where the contra lateral ear was examined and assessed for any hearing loss. Results: We found hearing loss in the contra lateral ear ranging from mild conductive hearing loss to sensorineural hearing loss with the maximum patients with mild conductive hearing loss (42%) and lowest in sensorineural hearing loss (1%). Conclusions: In our study, 76 patients were seen with conductive hearing loss. Out of that, 42% patients were seen with mild conductive hearing loss, 30% with moderate conductive hearing loss and 4% with severe conductive hearing loss. 20% patients were seen with normal hearing. 3% patients were seen with mixed hearing loss and only 1% patient was seen with sensorineural hearing loss in contralateral ear.

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Piezoelectric bone conduction hearing implant Osia® - audiological and quality of life benefits

Otolaryngologia Polska ◽

10.5604/01.3001.0014.9342 ◽

2021 ◽

Vol 75 (4) ◽

pp. 1-5

Author(s):

Joanna Marszał ◽

Renata Gibasiewicz ◽

Magdalena Błaszczyk ◽

Maria Gawlowska ◽

Wojciech Gawęcki

Keyword(s):

Quality Of Life ◽

Hearing Loss ◽

Hearing Aids ◽

Bone Conduction ◽

Speech Understanding ◽

Mixed Hearing Loss ◽

Wide Range ◽

The Mean ◽

Bone Conduction Hearing

Introduction: Nowadays, there are many options to treat hearing-impaired patients: tympanoplastic surgery, hearing aids and a wide range of implantable devices. Objective: The aim of this study is to present the mid-term audiological and quality of life benefits after the implantation of the Osia®, a new active piezoelectric bone conduction hearing implant. Material and methods: The state of the tissues in implanted area, as well as audiological and quality of life results were analyzed six, nine and twelve months after implantation in a group of four adult patients with bilateral mixed hearing loss. Results: In all the cases, no postoperative complications were found. One year after surgery the mean audiological gain in FF PTA4 (pure tone average for 0.5, 1, 2, and 4 kHz) was 52.2±3.5 dB in comparison to the unaided situation, the mean speech understanding with Osia® in quiet was 90±8.2% for 50dB SPL, 98.8±2.5% for 65dB SPL and 100±0% for 80dB SPL, and mean speech understanding with Osia® in noise was 37.5%±23.6 for 50dB SPL, 93.8±4.8% for 65dB SPL and 98.8±2.5% for 80dB SPL. There was also an evident improvement in the quality of hearing as well as in the quality of life, measured by the APHAB (Abbreviated Profile of Hearing Aid Benefit) and the SSQ (Speech, Spatial and Qualities of Hearing Scale). Conclusions: The Osia® is an effective treatment option for patients with bilateral mixed hearing loss. The mid-term audiological and quality of life results are excellent, but further observations including bigger groups of patients and a longer follow-up are required.

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Genetic Mixed Hearing Loss

Encyclopedia of Otolaryngology, Head and Neck Surgery ◽

10.1007/978-3-642-23499-6_100425 ◽

2013 ◽

pp. 992-992

Keyword(s):

Hearing Loss ◽

Mixed Hearing Loss

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