Objective: This case series aims to describe the diagnosis and management of otosclerosis in a series of Filipino patients encountered in a private, subspecialty otologic clinical practice. In particular, it elucidates the demographics, clinical presentation, diagnostic exam findings, clinical intervention and hearing outcomes. Methods: Study Design : Retrospective review of medical records of a case series. Setting : Subspecialty otologic / neurotologic clinical practice. Participants : Medical records of all patients diagnosed to have otosclerosis based on radiologic evidence of fenestral or retrofenestral otosclerosis and/or confirmed during surgical exploration of the middle ear, during the period 2004-2017. Results: Nine patients with otosclerosis were identified. Most patients presented with bilateral, primarily conductive hearing loss in middle age. However, an adolescent patient was also identified. Most patients were from the component cities of the National Capital Region. However, patients from the Cordillera region and south-central Mindanao were also identified. Radiologic evidence of fenestral or retrofenestral otosclerosis was identified in the majority of patients. However, readings of normal temporal bone CT findings were also seen. Obliterative otosclerosis was identified in one patient. Both objective and subjective evidence of hearing improvement was documented after stapedectomy in patients undergoing surgery. Conclusions This study documents the presence of clinical otosclerosis in a Filipino population. As in other populations, it typically presents in middle age as a bilateral, primarily conductive hearing loss. However, a younger age does not preclude its diagnosis, as juvenile otosclerosis has been identified. The presence of patients in regions of the Philippines other than the National Capital Region implies that it has to be considered even in regional populations without a racial predilection for otosclerosis. The identification of radiologic evidence of fenestral and retrofenestral otosclerosis on CT imaging of the temporal bone makes this is an essential part of the work-up of Filipino patients with conductive hearing loss and normal otologic examinations. Surgical treatment via stapes surgery is an effective and viable option in the management of Filipino patients with otosclerosis. The identification of less common variants such as obliterative otosclerosis indicates the need for specialized surgical equipment and appropriate surgical training in order to successfully deal with these situations.
Otosclerosis ; Hearing Loss, Conductive

A 29-year-old Filipina of Chinese descent presented with progressive bilateral conductive hearing loss of several years’ duration. While working overseas, she consulted with an otolaryngologist and underwent computerized tomographic (CT) imaging of the temporal bone as part of her evaluation. She was informed that no abnormalities were identified in the imaging exam, and she was offered exploratory middle ear surgery with possible stapes surgery. She then sought a second opinion, with the intention of obtaining a more definitive diagnosis prior to any invasive medical intervention. A review of the CT imaging study, with particular emphasis on looking for radiologic evidence of otosclerosis, revealed the presence of a focal region of bone demineralization in the region of the fissula ante fenestram. (Figure 1) This finding is consistent with a diagnosis of fenestral otosclerosis.
Otosclerosis ; Hearing Loss, Conductive ; Filipino

BACKGROUND AND OBJECTIVES: The purpose of the study was to compare the mastoid air-cell volume of the patients with superior semicircular canal dehiscence syndrome (SCDS) and that of the control patients with otosclerosis and temporal bone (TB) fracture. SUBJECTS AND METHODS: Ten patients with SCDS were enrolled and 10 patients with bilateral otosclerosis and TB fracture were selected as control groups by age and sex matching. To measure the mastoid air-cell volume, 3D reconstruction software was used. RESULTS: In 10 patients with SCDS, the mean age was 44.5 years, ranging from 16 to 79 years (M : F=4 : 6). Mean mastoid air-cell volume in the SCDS side was 3319.9 mm3, whereas 4177.2 mm3 in the normal side (p=0.022). Mean mastoid air-cell volume in the right side of otosclerosis patients was 6594.3 mm3 and it was not different from 6380.5 mm3 in the left side (p=0.445). Mean mastoid air-cell volume in normal side of TB fracture was 6477.2 mm3. The mastoid air-cell volume in the SCDS side was significantly smaller than that of otosclerosis and TB fracture patients (p=0.009, p=0.002, respectively). The mastoid air-cell volume in the normal side of SCDS was significantly smaller than that of TB fracture (p=0.019), but not significant with that of otosclerosis (p=0.063). CONCLUSIONS: Our findings revealed that the mastoid air-cell volume in the SCDS side was significantly smaller than control group, which suggest that the decreased mastoid pneumatization is closely related to the generation of SCDS.
Humans ; Mastoid ; Otosclerosis ; Semicircular Canals ; Temporal Bone

BACKGROUND AND OBJECTIVES: The purpose of the study was to compare the mastoid air-cell volume of the patients with superior semicircular canal dehiscence syndrome (SCDS) and that of the control patients with otosclerosis and temporal bone (TB) fracture. SUBJECTS AND METHODS: Ten patients with SCDS were enrolled and 10 patients with bilateral otosclerosis and TB fracture were selected as control groups by age and sex matching. To measure the mastoid air-cell volume, 3D reconstruction software was used. RESULTS: In 10 patients with SCDS, the mean age was 44.5 years, ranging from 16 to 79 years (M : F=4 : 6). Mean mastoid air-cell volume in the SCDS side was 3319.9 mm3, whereas 4177.2 mm3 in the normal side (p=0.022). Mean mastoid air-cell volume in the right side of otosclerosis patients was 6594.3 mm3 and it was not different from 6380.5 mm3 in the left side (p=0.445). Mean mastoid air-cell volume in normal side of TB fracture was 6477.2 mm3. The mastoid air-cell volume in the SCDS side was significantly smaller than that of otosclerosis and TB fracture patients (p=0.009, p=0.002, respectively). The mastoid air-cell volume in the normal side of SCDS was significantly smaller than that of TB fracture (p=0.019), but not significant with that of otosclerosis (p=0.063). CONCLUSIONS: Our findings revealed that the mastoid air-cell volume in the SCDS side was significantly smaller than control group, which suggest that the decreased mastoid pneumatization is closely related to the generation of SCDS.
Humans ; Mastoid ; Otosclerosis ; Semicircular Canals ; Temporal Bone

Otosclerosis occurs when the normal ivory-like endochondral bone of the otic capsule is replaced by foci of spongy vascular irregular new bone. Eventually these foci of haversian bone become more solid. There anr two typesof otosclerosis : fenestral and retrofenestral or cochlear. The cochlear type is much less common and rarely occurs without fenestral involvement. Otosclerosis occurs very rarely in blacks and orientals ; in otolaryngologic articles in Korea there are few reports of otosclerosis, and then only the fenestral type. This is a case reportof cochlear otosclerosis that can be diagnosed on temporal bone CT.
African Continental Ancestry Group ; Humans ; Korea ; Otosclerosis* ; Temporal Bone

BACKGROUND AND OBJECTIVES: A free-beam-type CO₂ laser, which use a micromanipulator mounted on a microscope as the delivery system, has the merit of not being affected by hand tremor at the time of shooting. However, this delivery system has several disadvantages, including a restricted operation range and a risk of incorrect focusing. A fiber-type CO₂ laser uses a hand-held delivery system and has the opposite merits and demerits. We compared the results of stapes surgery with free-beam and fiber type delivery systems. SUBJECTS AND METHODS: The study enrolled 36 patients who underwent stapedotomy with free-beam- (n=26) or fiber- (n=10) type CO₂ lasers. The air-bone (AB) gap closure, bone conduction (BC) change, and operating time were evaluated. The AB gap closure was calculated by subtracting the preoperative BC thresholds from the postoperative air conduction thresholds. The BC change was calculated by subtracting the postoperative BC thresholds from the preoperative BC thresholds. RESULTS: The mean operating time was significantly (p=0.035) shorter in the fiber-type group (72.5±8.2 min) than in the free-beam-type group (80.5±11.4 min). The mean AB gap closure did not differ significantly (p=0.297) between the free-beamand fiber-type groups (5.8±10.1 and 1.4±6.8 dB, respectively). The mean BC change did not differ significantly (p=0.873) between the free-beam- and fiber-type groups (2.4±6.9 and 2.8±5.3 dB, respectively). The hearing outcomes did not differ significantly between the two groups. CONCLUSIONS: Operating times were significantly shorter using the fiber-type CO₂ laser, while hearing outcomes did not differ significantly between the two groups.
Bone Conduction ; Hand ; Hearing ; Humans ; Otosclerosis ; Stapes Surgery* ; Stapes* ; Tremor

Otosclerosis is a primary metabolic bone disease of the otic capsule and ossicles. It is one of the causes of acquired hearing loss, with clinical manifestations occurring in approximately 1% of individuals in some populations in the Western countries. Although the cause of otosclerosis is undetermined, the disease has a well established hereditary predisposition, with approximately half of all affected individuals having family members known to be affected. Many genetic studies of otosclerosis support an autosomal dominant mode of inheritance with penetrance in the range of 20-40%. There have been a few reports of the clinically suspicious otosclerosis cases in Korea, but otosclerosis having familial forms have not been reported. We report one case of familial otosclerosis.
Bone Diseases, Metabolic ; Hearing Loss ; Humans ; Korea ; Otosclerosis* ; Penetrance ; Wills

BACKGROUND AND OBJECTIVES: Otosclerosis is known to be rare in orientals, but there have been a few reports of the clinically suspicious otosclerosis cases in Korea. Confirmation of otosclerosis, in theory, should be done by histopathology. However, for LASER stapedotomy, it is very hard to obtain the tissue to conduct such histopathological study. In this research, we had surgical specimens available for a review of otosclerosis case. We aimed to confirm the pathologic findings of otosclerosis in Koreans, and review their clinical information. SUBJECTS AND METHOD: Among 126 otosclerosis patients who were operated in the Seoul National University Hospital from January 1981 to December 2001, three cases were selected for pathologic evaluation. We used the H & E method for staining. RESULTS: There were positive histologic findings in all three cases. Two typical phases (early spongiotic and late sclerotic of otosclerosis) could be observed in specimen. CONCLUSION: In the pathologic specimen from the patients showing progressive conductive hearing loss, we could confirm otosclerosis. Both early and late phase of otsclerotic foci coexisted in the same specimen.
Hearing Loss, Conductive ; Humans ; Korea* ; Otosclerosis* ; Pathology ; Seoul

Humans ; Stapes ; Otosclerosis/surgery* ; Ossicular Prosthesis ; Stapes Surgery ; Incus

Humans ; Otosclerosis/surgery* ; Stapes ; Stapes Surgery/adverse effects* ; Arteries