Supralabyrinthine approach to petrosal cholesteatoma

Petrosal cholesteatomas are rare lesions, which may be congenital or acquired in nature. We report an exceptional case occurring in a seven-year old girl who presented with a unilateral conductive hearing loss, despite normal tympanic membrane appearance. Early diagnosis was facilitated by computed tomography (CT) scanning. Although this case satisfied the criteria for congenital cholesteatoma, it is likely that the petrous apex was secondarily involved. Complete cholesteatoma removal was accomplished using a transtemporal supralabyrinthine approach, which allowed for hearing preservation, while avoiding the morbidity associated with a craniotomy. The present case constitutes the youngest case of petrosal cholesteatoma reported. We suggest that a lower threshold for the use of CT scanning in unilateral conductive hearing loss may allow for the earlier detection of more cases of petrosal cholesteatomas, as well as facilitating their removal using more limited approaches associated with less morbidity.     

Temporal bone cholesteatoma

Clinical features of temporal bone cholesteatoma are miscellaneous, and sometimes misleading: signs of middle ear cholesteatoma, progressive or sudden facial palsy, sensorineural deafness as in acoustic neuroma, conductive deafness as in otosclerosis, secretory otitis media, or intracranial complications. Polytomography is the only way to pinpoint topography and extension. CT scanning is very useful in determining extension. Topography and severity of deafness are the guidelines for surgical approach. Among sixteen cases, total deafness was produced by the lesion itself in ten cases, and six had an intact inner ear; preservation of hearing was possible in only three. Supralabyrinthine cholesteatomas (five cases) are best managed by the middle fossa approach. Infralabyrinthine cholesteatomas (six cases) must be removed via the infratemporal approach with anterior displacement of the tympanic and mastoid segments of the facial nerve and permanent obliteration of the middle ear. Posterior perilabyrinthine cholesteatoma (five cases) may be removed by the otologic approach combined with the middle ear fossa approach if the inner ear is to be preserved. If hearing loss is total, the translabyrinthine approach can be used.     

Congenital Cholesteatomas in the Tympanic Membrane

The etiology of congenital middle ear (ME) cholesteatomas is unclear. One etiologic possibility of ME cholesteatoma may be progression of a congenital tympanic membrane (TM) cholesteatoma. We recently have encountered three cases of congenital tympanic membrane cholesteatoma. Each child, ages 1, 3, and 14 years, presented with cholesteatoma of the tympanic membrane extending into the middle ear. These children have not had previous otologic surgery including myringotomy, nor had they had repeated middle ear infections, perforation, or trauma. Neither the 3-year-old nor 14-year-old child complained of hearing loss. Audiograms demonstrated only a mild conductive loss. Each child underwent excision with tympanoplasty. Although the middle ear component of the cholesteatoma was always more extensive than the pearl seen, the point of attachment was the TM and not the middle ear. This demonstrates one possible source for congenital cholesteatomas.     

Clinical evaluation of congenital cholesteatoma of the middle ear

We conducted a retrospective study to identify the clinical features and surgical observations of congenital cholesteatoma. Sixty patients were diagnosed and underwent surgery for congenital cholesteatoma between April 1987 and May 2002. All diagnoses were made on the basis of two operative findings: 1. the tympanic membrane manifested neither retraction, perforation, nor granulation. 2. the tympanic membrane was not continuous with the cholesteatoma. In this series, congenital cholesteatoma accounted for 7% of all cholesteatomas (853 ears). The patient age ranged from 2 to 48 years. The male to female ratio was 4:1. Seventeen patients had multiple cholesteatoma. Fifty-three patients exhibited closed-type cholesteatomas, while the remaining 7 patients had open-type cholesteatomas that had formed as a flat surface of the epidermis. Patients with open-type cholesteatomas presented with a much more pronounced conductive hearing loss and ossicular erosion or malformation. Twenty-two patients with relatively small cholesteatomas were analyzed to estimate the origin of their cholesteatomas. Of the 22 patients, 13 had anterior superior quadrant (ASQ-type) and 9 had posterior superior quadrant (PSQ-type) cholesteatomas. The mean age at the time of detection was older in the PSQ-type group than in the ASQ-type group and the frequency of ossicular erosion or malformation was more prominent in the PSQ-type group than in the ASQ-type group. The primary site of origin was thought to be the portion between the tympanic ostium of the auditory canal and the semicanal for tensor tympani in the ASQ-type group and near the incudostapedial joint in the PSQ-type group. A planned staged procedure was performed in 29 patients, 15 patients (52%) had residual lesions situated mostly on the oval window, the round window, an exposed facial nerve or an exposed lateral semicircular canal. The frequency of residual lesions in patients who presented with extended, multiple cholesteatoma and those with ossicular malformation was comparable to the frequency of patients who did not present with these features.     

Congenital cholesteatoma: classification,management, and outcome

OBJECTIVES: To assess whether a classification system for congenital cholesteatoma (CC) can be derived from analysis of a large clinical sample of cases and to assess whether such a classification system is a reliable guide for surgical intervention, reexploration, and hearing outcome. DESIGN: A retrospective review of clinical and surgical records of 119 patients with CC. SETTING: Four tertiary care children's hospitals. PATIENTS: One hundred nineteen children with CC (age range, 2-14 years). RESULTS: Congenital cholesteatomas in the anterior mesotympanum were treated successfully with exploratory tympanotomy. Congenital cholesteatomas involving the posterior superior quadrant and the attic usually had concurrent involvement of the incus and stapes and often required a canal wall up tympanomastoidectomy and a second look for its control. Congenital cholesteatoma involving the mastoid usually involved all of the ossicles, was inconsistently controlled with canal wall up tympanomastoidectomy, and had a poor prognosis for restoration of conductive hearing loss. The mean +/- SD age of children with CC was 5.6 +/- 2.8 years, while that of children with acquired cholesteatoma was 9.7 +/- 3.3 years. CONCLUSIONS: The sequence of spread of CC, involving 3 sites, suggests a natural classification system. The CC usually originates in the anterior superior quadrant, but does not consistently remain there, and may variably occupy the middle ear and mastoid and result in ossicular destruction and conductive hearing loss. The location of CC and the involvement of the ossicles is an accurate predictor of the type of surgery necessary for its control and for the success of hearing restoration.     

Fibrous dysplasia of the temporal bone

A 38-year-old woman with fibrous dysplasia of the entire right half of the mandible and the right temporal bone is presented. She had a unilateral progressive conductive hearing loss and stenosis of the auditory canal complicated by a chronic external otitis, a hidden cholesteatoma, destruction of the ossicles and an impending facial palsy. Surgery was performed to create a new auditory canal, to eradicate the cholesteatoma and to decompress the facial nerve from the surrounding fibrous dysplasia.     

Petrous cholesteatoma: clinical features and surgical outcome

In this study we analyzed the clinical features and surgical treatment of petrous cholesteatoma in 9 patients (9 ears) who underwent surgery at the Jikei University Hospital. Most patients initially experienced hearing loss, then facial nerve palsy or otorrhea. Congenital cholesteatoma was diagnosed in four patients, acquired cholesteatoma in one patient, and postoperative recurrence in three patients. The proportion of cases of congenital cholesteatomas was higher than in previous reports. The area of the cholesteatoma was classified according to Dr. Sanna cases and the supralabyrinthine type in two cases. Hearing preservation surgery was performed in four patients via the middle cranial fossa approach or by partial labyrinthectomy, and the results seemed satisfactory. These results suggested that hearing preservation surgery should be adopted in every case.     

Mechanisms of cholesteatoma formation following stapedectomy

Poststapedectomy cholesteatoma is uncommon. We report a patient with bilateral cholesteatomas, each following an initially successful stapedectomy for conductive hearing loss due to stapedial fixation from otosclerosis. A revision right stapedectomy done in 1972 was complicated 4 years later by prosthesis extrusion and cholesteatoma. An extensive left cholesteatoma occurred 7 years after stapedectomy in 1977 and required a radical mastoidectomy. The etiology of poststapedectomy cholesteatoma in this patient was probably chronic eustachian tube dysfunction and negative middle ear pressures. The initial right cholesteatoma occurred following prosthesis extrusion, which is presumed to have been the portal of entry of squamous epithelium into the middle ear. The later-occurring left poststapedectomy cholesteatoma represented progression of an attic retraction pocket. Both of these complications were likely secondary to negative middle ear pressure. In addition to eustachian tube dysfunction, other proposed etiologies for poststapedectomy cholesteatoma formation include prosthesis extrusion independent of negative middle ear pressure, unrecognized presence of squamous epithelium in an oval window fat graft, an inverted tympanomeatal flap due to improper positioning, and a marginal perforation from a disrupted anulus. Poststapedectomy cholesteatoma is a rare phenomenon, but does occur. The preoperative exclusion of patients with eustachian tube dysfunction and proper surgical techniques should reduce this complication.     

Management of pediatric cholesteatoma based on presentations,complications, and outcomes

ObjectivesTo highlight important aspects and paradigms in the management of paediatric cholesteatoma in a developing world setting.MethodsA retrospective audit was conducted of paediatric cholesteatomas that underwent tympanomastoid surgery between 2008 and 2012 at the Red Cross War Memorial Children's Hospital in Cape Town. The following was audited: initial presentation; cholesteatoma complications; types of surgery, intraoperative findings and outcomes of surgery in terms of hearing, otorrhoea and recidivism; and the reliability of follow-up and how this might influence the type of surgery.ResultsFifty-seven children aged 2–13 years with 61 cholesteatomas (4 bilateral) were reviewed. Fifty-five mastoidectomies were done; 11% presented with complicated cholesteatoma. Referrals from primary care were significantly delayed (>6 months) in 76%. Canal wall down surgery was done in 71%. Forty-five percent had improved hearing (within 15 dB of better hearing ear) and a further 15% had no or only mild hearing loss. Ossicular chain involvement and ossicles encased in inflammatory tissue were associated with poorer hearing outcomes. Sixty-four percent of ears remained dry. Forty-five percent of the canal wall up, and 23% of canal wall down mastoidectomies had recidivism. Twenty-six percent of patients were lost to follow-up.ConclusionsChildren are likely to present with advanced cholesteatoma with ossicular chain involvement. The children present with high rates of complications, poor pre-operative hearing and have high recurrence rates post-surgery. Referral from primary health care is delayed. Canal wall down procedures may be appropriate in a setting where patient follow-up is unreliable and access to operating theatre is limited.     

Congenital cholesteatoma.

We present a British series of eleven patients with primary cholesteatoma, including one patient with bilateral disease. Eight children presented with a history of hearing loss, while one child had had recurrent otitis media and another had had earache. Operative findings were: in five ears, cholesteatoma confined to the antero-superior segment with intact ossicles, in a further four, cholesteatomas extending throughout the mesotympanum with ossicular erosion in one, and in two ears posterior disease throughout the middle ear and mastoid, which had eroded the ossicles in both cases. The five cases of antero-superior cholesteatoma lend most support to Michaels' concept of epidermoid formation as a possible source of congenital cholesteatoma. With a greater awareness of the problem and careful examination of the antero-superior quadrant of the tympanic membrane, earlier diagnosis may be possible enabling removal of small intact cholesteatoma sacs and preserving the structures of the middle ear and therefore the hearing. A screening programme for infants included as part of their routine examination which would be undertaken by examiners who are trained to be more aware of the problem and skilled at otoscopy, would help in the earlier detection of such cases as is shown by reports from the U.S.A.     

10例先天性中耳胆脂瘤临床分析

目的：探讨先天性中耳胆脂瘤的临床特征及手术方法。方法：回顾性分析10例先天性中耳胆脂瘤患者的临床资料。5例经耳内途径行鼓室探查并一期鼓室成形术,3例行闭合式乳突根治术及鼓室成形术,2例行开放式乳突根治术及鼓室成形术。结果：术中见5例胆脂瘤局限于中后鼓室,3例位于中鼓室及上鼓室,2例病变范围广泛,侵及乳突。术后6个月平均纯音听阈为30dBHL,气骨导差在20dB以内,复查颞骨CT均未发现胆脂瘤残留和复发。结论：先天性中耳胆脂瘤病变隐匿,常于鼓室前方或后方,易破坏听骨链,导致传导性聋;影像学检查可为诊断及术式选择提供依据;早期手术治疗可获得较好的听力重建效果。     

Petrous apex cholesteatoma: Diagnostic and treatment dilemmas

The diagnosis and treatment of petrous apex cholesteatoma is a difficult surgical challenge. This study is a review of 14 cases of cholesteatoma involving the petrous apex. These cholesteatomas originated as a congenital primary lesion or secondary to an acquired lesion. The cases were evaluated according to the clinical features, the intraoperative findings, the radiological findings, and the surgical approaches. In this series, 83% of the patients presented with hearing loss and 50% presented with facial nerve weakness or paralysis ←House grade II to VI→. Intraoperative and radiological features revealed frequent direct labyrinthine and supralabyrinthine cell spread. The transpetrous surgical approach was used in all cases. The main factors affecting the surgical approach to be adopted are the inaccessible nature of the petrous apex, the extent of disease, the degree of facial nerve function, and the need for the prevention of cerebrospinal fluid leaks and the recurrence of the lesion.     

Epidemiology of middle ear and mastoid cholesteatomas. Study of 1146 cases

Middle ear cholesteatoma is an important and relatively common disorder which may have serious consequences.AimThe purpose was to conduct a retrospective study of the statistics of 1,146 middle ear surgical procedures for middle ear cholesteatoma in adults and children of low income living in distant areas from our city.MethodsFrom 1962 to 1988 there were 1,146 surgeries for unilateral or bilateral cholesteatomas in children and adults, which were reviewed for the following criteria: total number of surgeries, sex, onset of the first symptoms, duration of the disease, the site of perforation, the cholesteatoma site, changes in the ossicular chain, the contralateral ear, bilateral cholesteatomas, the site of residual cholesteatoma, and complications.ResultsResults are shown graphically on a pie chart.ConclusionThe etiology of cholesteatomas remains unknown. Epidemiological and statistical data, surgical reports, and conclusions of experimental studies are welcome, as they may provide support for clarifying the pathogenesis of cholesteatoma. Our results were compared with internationally published papers. We found no published papers on the epidemiology of cholesteatoma in the Brazilian literature.     

Congenital middle ear cholesteatoma: report of 3 cases.

In this report, we presented 3 cases of congenital middle ear cholesteatoma which occurred in a 12-year-old girl, a 4-year-old boy, and a 6-year-old boy. In all 3 cases, there was a whitish mass behind a normal tympanic membrane. Congenital middle ear cholesteatoma is not a rare disease. In the early stage, it is asymptomatic. But when it progresses, this disorder can destroy conductive systems of the middle ear and cause many symptoms. One patient (Case 1) had a complaint of hearing impairment. She underwent mastoidectomy and tympanoplasty; however, the cholesteatoma recurred. The other 2 patients had no symptoms. The abnormal appearance of their tympanic membrane was found by chance at their local otologists. We performed tympanotomies and removed cholesteatomas without aftereffects. When otologists note an abnormal appearance behind a normal tympanic membrane, with or without symptoms, tympanotomy should be done due to the possibility of congenital middle ear cholesteatoma.     

Treatment of sinus cholesteatoma. Long-term results and recurrence rate

We classified cholesteatomas as attic cholesteatoma, developing from Shrapnell's membrane; tensa cholesteatoma, originating in pars tensa, which is subdivided into tensa retraction cholesteatoma involving the entire pars tensa, and sinus cholesteatomas, developing from a posterosuperior retraction (perforation). From 1964 to 1980, one-stage operations were carried out on 271 ears with sinus cholesteatomas. Follow-up included 90% of the patients, and the median observation time was 9.75 years. The recurrence rate was 10%. The recurrence rate was found to be independent of the mastoidectomy type employed. The best hearing results were obtained in ears with intact ossicular chain. We conclude that, wherever possible, sinus cholesteatoma should be removed through the auditory canal without mastoidectomy just as an intact ossicular chain should be preserved. "Canal wall up" and "canal wall down" appear to be equally valuable mastoidectomy types, and both methods must be employed to obtain optimum results.     

Spontaneous cholesteatoma of the external auditory canal

Spontaneous cholesteatoma of the external auditory canal is a rare disease which may also be under diagnosed. Few cases have been reported even in major otology centers. Possible theories as to the aetiology are based on observation of these few cases. The correct diagnosis allows the proper treatment avoiding the progression of the cholesteatoma and further hearing loss or other complications. The authors review five cases and discuss the possibility that it is the anatomic structure of the bony external auditory canal bone that might contribute to the etiopathogenesis of these cholesteatomas.     

Development of canal cholesteatoma in a patient with prenatal isotretinoin exposure

Purpose

To describe the clinical and radiologic findings in a case of isotretinoin embryopathy-like syndrome and discuss management of hearing loss, congenital external auditory canal (EAC) stenosis, and EAC cholesteatoma.

Methods

Review of medical, audiological, and radiological records.

Results

An 8 year old female presented with bilateral moderate conductive hearing loss, bilateral microtia, left EAC stenosis, and right EAC atresia, secondary to prenatal isotretinoin exposure. Comorbidities included developmental delay, ventricular septal defect, hypotonia, and retinal maldevelopment. The left EAC was sharply upsloping with a 2 mm-diameter meatus. Computed tomography (CT) scan of the temporal bone demonstrated normal middle and inner ears bilaterally; serial CT scans over 6 years demonstrated progressive development of left canal cholesteatoma. Implantation of a right BAHA system was performed, followed by left canalplasty and excision of cholesteatoma with facial nerve monitoring. An endaural incision was utilized to avoid compromising future microtia repair. Postoperative left-sided hearing improved to mild low-frequency conductive hearing loss rising to normal at 2000 Hz and above.

Conclusions

Despite extensive precautions for its use, isotretinoin remains a cause of major birth defects, including sensorineural, conductive or mixed hearing loss. Congenital EAC stenosis is much less common than congenital atresia or acquired stenosis; optimal surgical approaches vary depending on hearing status and facial nerve anatomy. Close monitoring for development of canal cholesteatoma is necessary.     

Late results of surgery in different cholesteatoma types

Our series of 740 cholesteatomas, operated during the period 1969 to 1980, were seen at follow-up several times with a median observation period of 9.2 years (range 3-21 years). There were; 273 attic cholesteatomas with retraction (perforation) of Shrapnell's membrane; 271 sinus cholesteatomas with superioposterior retraction (perforation) of pars tensa, and 196 tensa retraction cholesteatomas extending from a retraction of the whole pars tensa. The late results were analyzed for each type separately, and compared. The recurrence rate was lowest (6.6%) in attic cholesteatoma and highest (13.3%) in tensa retraction cholesteatoma. In all three types no residual cholesteatomas were detected after the 4th postoperative year, whereas recurrent cholesteatomas occurred up to 10 years after surgery. The reoperation rate was lowest (15%) in attic cholesteatoma and almost the same (21%) in sinus and tensa retraction cholesteatoma. The hearing results were best in attic cholesteatoma and poorest in tensa retraction cholesteatoma. It is concluded that cholesteatoma surgery should be individualized and that both the canal wall up and canal wall down methods have their place in cholesteatoma surgery.     

Cholesteatoma in children

Of 325 previously untreated cholesteatomas, 109 were found to be in children 13 years old and younger--however, these 109 ears were not found to consist of one clinical entity. Sixty-three of the 109 ears presented a marginal perforation or a retraction pocket, at the level of Shrapnell's membrane or beyond the postero-superior quadrant. In this group the cholesteatoma was distributed mainly in the attic and mastoid and was associated with a non-cellular mastoid. A second group comprising 31 ears presented with cholesteatoma behind an intact drum and were considered to be primary cholesteatomas. These were distributed mostly in the tympanic cavity as cystic epidermoid formations--their mastoid was usually pneumatized. Eight cholesteatomas were related to a central perforation. These ears presented features very similar to the primary cholesteatomas i.e., a pneumatized mastoid and tympanic cavity distribution. The similar features of this group make us think that they may have also originated as primary cholesteatomas which eventually perforated and bring the percentage of primary cholesteatomas in children to 38.3%. Seven of the 109 ears with cholesteatoma were of an indeterminate character.     

Surgical treatment of pediatric cholesteatomas

OBJECTIVE:: Management of pediatric cholesteatomas remains controversial. We reviewed our 16-year experience in the surgical treatment of cholesteatomas in children and describe a treatment paradigm. STUDY DESIGN:: The authors conducted a retrospective review. METHODS:: A total of 106 mastoidectomies (86 for an acquired cholesteatoma and 20 for a congenital cholesteatoma) were performed in children 16 years old and younger from 1988 to 2003. Follow up ranged from 2 years to 12 years with a mean follow-up period of 6 years. Hearing outcomes, cholesteatoma recidivism, and dry mastoid cavity were the main outcomes measured. RESULTS:: Seven (7%) patients had revision surgery for cholesteatoma recidivism. Rates of cholesteatoma recurrence for canal all up (CWU) and canal wall down (CWD) mastoidectomy groups were similar (8% vs. 6%). The percentage of patient with good serviceable hearing (pure-tone average 相似文献