Ehlers-Danlos Syndrome: Aural manifestations and treatment

Thomas, D. M.; Wright, J. L. W.; Soucek, Sava; Shalom, A.

doi:10.1016/s0196-0709(96)90081-2

Cited by 6 publications

(9 citation statements)

References 5 publications

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“…In 21 patients with EDS-HT, Castori, et al found that 23.8% of the patients subjectively reported mild hearing impairment [6]. However, only three patients with EDS have been reported in the literature to develop significant hearing loss [7-9]. In two patients, their conductive hearing loss was attributed to otosclerosis, while the third was attributed to retraction of the tympanic membrane.…”

Section: Discussionmentioning

confidence: 99%

Superior Semicircular Canal Dehiscence in a Patient with Ehlers-Danlos Syndrome: A Case Report

Chung¹,

Lagman²,

Nagasawa³

et al. 2017

Cureus

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Superior semicircular canal dehiscence (SSCD) is a bony defect in the middle cranial fossa floor that results in an abnormal connection between the inner ear and cranial vault. Although the etiology of SSCD remains unclear, an inappropriately thin or fragile temporal bone likely predisposes an individual towards developing SSCD. Ehlers-Danlos syndrome (EDS) constitutes a group of genetic connective tissue disorders caused by a defect in the production, processing, or structure of collagen, or its associated proteins. The possible association between SSCD and EDS has not been previously described in the literature. We herein report a case of a 50-year-old female with EDS-hypermobility type who presented with a 15-year history of migraines, vertigo, and tinnitus. The patient was subsequently diagnosed with bilateral SSCD and underwent a right middle fossa (pre-auricular infratemporal) craniotomy for SSCD repair. She reported significant improvement in her auditory and vestibular symptoms, with the exception of continued mild dizziness and disequilibrium at the 3-month follow-up. Due to the rare reports of auditory symptoms in EDS, this case study highlights the importance of considering an otological consultation for auditory manifestations in a patient with EDS and illustrates a potential association between EDS and SSCD.

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Section: Discussionmentioning

confidence: 99%

Superior Semicircular Canal Dehiscence in a Patient with Ehlers-Danlos Syndrome: A Case Report

Chung¹,

Lagman²,

Nagasawa³

et al. 2017

Cureus

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“…The recurrent facial palsy following stapedotomy in a case reported by Mair et al [2] and occurrence of acute and chronic inflammatory granulation tissues following insertion of a ventilation tube in a case reported by Thomas et al [3] strongly suggest that placement of foreign bodies such as a Teflon piston wire is contraindicated in patients with EDS. Our patient underwent stapes mobilization together with removal of scar tissues around the ossicles instead, after which hearing was markedly improved.…”

Section: Discussionmentioning

confidence: 99%

“…Mair et al [2] reported a 27-year-old female with hearing loss due to otosclerosis, who underwent a successful stapedotomy but exhibited recurrent facial palsy postoperatively. Thomas et al [3] reported a 62-year-old man with conductive hearing losses due to retraction of the tympanic membrane in both ears. This patient underwent insertions of Shah aural grommets in both ears, but his hearing deteriorated due to the accumulation of effusions.…”

Section: Discussionmentioning

confidence: 99%

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Otosclerosis associated with Ehlers-Danlos syndrome: report of a case

Miyajima

Ishimoto

Yamasoba

2007

Acta Oto-Laryngologica

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Hearing loss occurs rarely in Ehlers-Danlos syndrome (EDS). We report a case of a 24-year-old woman with EDS who had conductive deafness due to otosclerosis and scar tissues around the malleus and incus. The scar was considered to develop by inflammatory response specific to EDS that had been induced by otitis media with effusion. Following stapes mobilization and removal of the scar tissues, her hearing was improved for several months but showed deterioration thereafter, probably due to recurrence of ossicular fixation. The tendency toward scar formation, infection, and inflammatory response, especially to foreign bodies, in EDS needs to be kept in mind to determine treatment of choice in patients with EDS and conductive hearing loss.

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“…The findings of CHL, MHL, or SubC component in 53% (19 patients) of our cohort also could be associated with connective tissue changes within the TM or the ligaments around the middle ear ossicles, as seen in other connective tissue disorders such as Ehlers-Danlos syndrome and Stickler syndrome. 12,13…”

Section: Discussionmentioning

confidence: 99%

Audiologic and Otologic Clinical Manifestations of Loeys‐Dietz Syndrome: A Heritable Connective Tissue Disorder

Jeon

Christensen

Chisholm

et al. 2021

Otolaryngol.--head neck surg.

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Objective Loeys-Dietz syndrome (LDS) is a rare genetic connective tissue disorder resulting from TGF-ß signaling pathway defects and characterized by a wide spectrum of aortic aneurysm, arterial tortuosity, and various extravascular abnormalities. This study describes the audiologic, otologic, and craniofacial manifestations of LDS. Study Design Consecutive cross-sectional study. Setting Tertiary medical research institute. Methods Audiologic and clinical evaluations were conducted among 36 patients (mean ± SD age, 24 ± 17 years; 54% female) with genetically confirmed LDS. Cases were categorized into genetically based LDS types 1 to 4 ( TGFBR1, TGFBR2, SMAD3, TGFB2, respectively). Audiometric characteristics included degree and type of hearing loss: subclinical, conductive, mixed, and sensorineural. Results LDS types 1 to 4 included 11, 13, 5, and 7 patients, respectively. In LDS-1, 27% had bilateral conductive hearing loss; 9%, unilateral mixed; and 36%, subclinical. In LDS-2, 38% had conductive hearing loss and 38% subclinical. In LDS-3 and LDS-4, 40% and 43% had bilateral sensorineural hearing loss, respectively. Degree of hearing loss ranged from mild to moderate. Bifid uvula was observed only in LDS-1 (55%) and LDS-2 (62%). Submucosal/hard cleft palates were primarily in LDS-1 and LDS-2. Posttympanostomy tympanic membrane perforations occurred in 45% (10/22 ears) of LDS-1 and LDS-2. There were 4 cases of cholesteatoma: 3 middle ear (LDS-1 and LDS-2) and 1 external ear canal (LDS-3). Conclusion Conductive hearing loss, bifid uvula/cleft palate, and posttympanostomy tympanic membrane perforation are more common in LDS-1 and LDS-2 than LDS-3 and LDS-4, while sensorineural hearing loss was present only in LDS-3 and LDS-4. These LDS-associated key clinical presentations may facilitate an early diagnosis of LDS and thus prompt intervention to prevent related detrimental outcomes.

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Ehlers-Danlos Syndrome: Aural manifestations and treatment

Cited by 6 publications

References 5 publications

Superior Semicircular Canal Dehiscence in a Patient with Ehlers-Danlos Syndrome: A Case Report

Superior Semicircular Canal Dehiscence in a Patient with Ehlers-Danlos Syndrome: A Case Report

Otosclerosis associated with Ehlers-Danlos syndrome: report of a case

Audiologic and Otologic Clinical Manifestations of Loeys‐Dietz Syndrome: A Heritable Connective Tissue Disorder

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