Pediatric Otosclerosis
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Otosclerosis is a genetically mediated metabolic bone disease that affects only the human otic capsule and ossicles. [1, 2] Its mode of inheritance is autosomal dominant, but penetrance and expressivity both vary.
Usually, symptomatic hearing loss from otosclerosis first develops early in the third decade of life, though onset can occur during childhood. Otosclerosis is well recognized as a cause of conductive hearing loss from fixation of the stapedial footplate in the oval window niche. Less well recognized is that otosclerosis can involve other portions of the cochlea and produce sensorineural hearing loss (SNHL).
The incidence of cochlear otosclerosis and that of cochlear otosclerosis resulting in clinically significant SNHL are unclear. The disease occurs in 8-10% of the white population; however, of those individuals affected, only 10-15% have clinical symptoms. Consequently, conductive hearing loss that necessitates treatment eventually develops in about 1% of the white population. Clinical disease occurs in about 0.5% of people of Asian and South American descent and in about 0.1% of those of African descent. The disease is bilateral in most cases.
For patient education resources, see the Ear, Nose, and Throat Center, as well as Tinnitus.
Two separate pathologic phases of the disease process can be identified:
The otosclerotic phase begins when osteoclasts are slowly replaced by osteoblasts and dense sclerotic bone is deposited in the areas of previous bone resorption. When this process involves the oval window in the area of the footplate, the footplate becomes fixed, resulting in conductive hearing loss. [3] (See Histologic Findings.)
Histologically, otosclerosis has a prevalence of about 10%. However, only about 10% of persons with histologic disease are clinically affected, and thus, the prevalence of clinically significant disease is about 1%. The most common morbidity from otosclerosis is hearing loss. Although conductive hearing loss is considered the hallmark of the disease, involvement of portions of the otic capsule other than the stapedial footplate can result in SNHL. Rarely, patients may present with vertigo. [4]
Clinical otosclerosis can manifest as early as age 7-8 years but most commonly appears in persons aged 15-35 years. Women seek medical attention for hearing loss due to otosclerosis more frequently than men do. However, the disease is not sex-linked, and a histologic study of a large series of temporal bones shows no difference in prevalence between men and women. Otosclerosis is much more common in whites than in persons of other races: Histologic otosclerosis occurs in 10-20% of whites but in only about 1% of people of African descent.
The clinical progression of the disease is unpredictable. Bilateral involvement is typical, and, in most cases, conductive hearing loss progresses until the maximal 50-60 dB loss develops. SNHL can result in total deafness, but such severe progression is uncommon.
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Bittermann AJ, Rovers MM, Tange RA, Vincent R, Dreschler WA, Grolman W. Primary stapes surgery in patients with otosclerosis: prediction of postoperative outcome. Arch Otolaryngol Head Neck Surg. 2011 Aug. 137 (8):780-4. [Medline].
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Neilan RE, Zhang RW, Roland PS, Isaacson B, Lee KH, Walter Kutz J Jr. Pediatric stapedectomy: does cause of fixation affect outcomes?. Int J Pediatr Otorhinolaryngol. 2013 Jul. 77 (7):1099-102. [Medline].
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Joe Walter Kutz, Jr, MD, FACS Associate Professor, Associate Residency Director, Neurotology Fellowship Director, Department of Otolaryngology–Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas, Southwestern Medical School
Joe Walter Kutz, Jr, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American Neurotology Society, Otosclerosis Study Group, Texas Medical Association, Triological Society
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Achaogen; Eloxx<br/>Received research grant from: Otonomy; Auris Medical<br/>Received income in an amount equal to or greater than $250 from: Medtronic.
Peter S Roland, MD Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director, Clinical Center for Auditory, Vestibular, and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Chief of Pediatric Otology, Children’s Medical Center of Dallas; President of Medical Staff, Parkland Memorial Hospital; Adjunct Professor of Communicative Disorders, School of Behavioral and Brain Sciences, Chief of Medical Service, Callier Center for Communicative Disorders, University of Texas School of Human Development
Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Neurotology Society, American Otological Society, North American Skull Base Society, Society of University Otolaryngologists-Head and Neck Surgeons, The Triological Society
Disclosure: Received honoraria from Alcon Labs for consulting; Received honoraria from Advanced Bionics for board membership; Received honoraria from Cochlear Corp for board membership; Received travel grants from Med El Corp for consulting.
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
John E McClay, MD Associate Professor of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Children’s Hospital of Dallas, University of Texas Southwestern Medical Center
John E McClay, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association
Disclosure: Nothing to disclose.
Ravindhra G Elluru, MD, PhD Professor, Wright State University, Boonshoft School of Medicine; Pediatric Otolaryngologist, Department of Otolaryngology, Dayton Children’s Hospital Medical Center
Ravindhra G Elluru, MD, PhD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, Association for Research in Otolaryngology, Society for Ear, Nose and Throat Advances in Children, Triological Society, American Society for Cell Biology
Disclosure: Nothing to disclose.
Orval Brown, MD Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas
Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Nothing to disclose.
Pediatric Otosclerosis
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