Spontaneous Resolution of Cholesteatoma in a Patient on Long-Term Infliximab.

OBJECTIVE:: To describe an observed case of spontaneous regression of cholesteatoma in a patient on chronic anti-tumor necrosis factor-alpha (TNF-a) therapy and inspire further research into the role of TNF-a in cholesteatoma. METHODS:: Clinical assessment of disease in a single-patient case report. RESULTS:: A 49-year-old woman suffered a severe case of Stevens-Johnson syndrome when she was 12 years old, leaving her with bilateral corneal opacification and tympanic membrane perforations with extensive cholesteatoma. For her corneal opacification, a corneal prosthesis was placed, which was complicated by a foreign body reaction necessitating long-term therapy with infliximab, a monoclonal antibody against TNF-a that is therapeutic in some chronic inflammatory diseases. She was otherwise healthy and took no other medications. While on infliximab, the patient had spontaneous and complete resolution of her cholesteatoma without any surgical intervention. CONCLUSIONS:: This surprising case suggests that there may be a prominent role of TNF-a in cholesteatoma pathophysiology and that TNF-a may be an effective target for nonsurgical therapy.

Laser-induced tissue remodeling within the tympanic membrane.

The tympanic membrane (TM) separates the outer ear from the tympanic cavity. Repeated pathologies can permanently decrease its tension, inducing conductive hearing loss and adhesive processes up to cholesteatoma. The current main therapy is its surgical reconstruction. Even though lasers have been proposed to tighten atrophic TMs, details of this effect, specifically histological analyses, are missing. We therefore used laser pulses to induce TM collagen remodeling in an animal model to compare the histological and electrophysiological effects of different applied laser intensities before entering clinical studies. We irradiated Fuchsin-stained areas of the TM in anesthetized mice with 532-nm laser-pulses of 10 mW for 30 s (0.3 J), 25 mW for 30 s (0.75 J) or 50 mW for 30 s (1.5 J) monitoring hearing with auditory brainstem responses (ABRs). The mice were sacrificed after 2 to 8 weeks and histologically analyzed. An increase in the TM thickness within the defined, stained, and irradiated areas could be observed after 4 weeks. Polarized light microscopy and transmission electron microscopy demonstrated the tissue volume increase majorly due to new collagen-fibrils. Directly after irradiation, ABR thresholds did not increase. We herein demonstrate a controlled laser-induced collagen remodeling within defined areas of the TM. This method might be the prophylactic solution for chronic inflammatory ear pathologies related to decreased TM tension.

[Retrospective clinical analyses of otogenic intracranial infections].

Objective: To investigate the clinical characteristics and treatment options for otogenic intracranial infections. Method: Clinical records of all the patients of otogenic intracranial infections admitted to the First Affiliated Hospital of Zhejiang University from 2008 to 2016 were retrospectively analyzed. Their clinical presentations, radiological findings, culture results, and medical and surgical therapy modalities, as well as treatment outcomes were studied. Results: Sixteen cases were identified. The majority of the otogenic patients had a history of cholesteatoma, other rare events included congenital cerebrospinal fluid otorhinorrhea, Gorham-Stout disease and after radiation therapy for nasopharyngeal carcinoma. Meningitis, cerebral venous thrombosis and brain abscess were the primary intracranial infection. Eight patients had received modified radical mastoidectomy at least one time. Results of routine culture for cerebrospinal and pus samples had high negative rate. All patients received initial empirical broadspectrum intravenous antibiotics therapy. Four cases of brain abscesses were drained or excised at the same time for otologic surgery. The mortality rate was 6.25% (1 case). Conclusions: Cholesteatoma is still the most commonly primary disease of otogenic intracranial complications. Diagnosis and treatment of otogenic intracranial infections require multidisciplinary cooperation. Surgical intervention for primary ear lesions and intracranial abscess is still the main option in the treatment of otogenic intracranial infections.

External auditory canal cholesteatoma and benign necrotising otitis externa: clinical study of 95 cases in the Capital Region of Denmark.

OBJECTIVE: To identify epidemiological and pathophysiological factors, and treatment strategies, in external auditory canal cholesteatoma and benign necrotising otitis externa. METHODS: A retrospective case study was conducted of patients suffering from external auditory canal cholesteatoma and benign necrotising otitis externa admitted to tertiary hospitals, in the Capital Region of Denmark, over a five-year period. RESULTS: Eighty-three patients (95 ears) with external auditory canal cholesteatoma or benign necrotising otitis externa were identified. A minimum incidence rate of 0.97 per 100 000 inhabitants per year was demonstrated. Sixty-eight per cent of cases had a history of smoking. Most lesions (74 per cent) were localised in the floor of the ear canal. Treatment time was 3.2 months for patients who had surgery and 6.0 months for those who received conservative treatment. CONCLUSION: It is suggested that external auditory canal cholesteatoma and benign necrotising otitis externa are in fact the same disease, and therefore the diagnosis of external auditory canal cholesteatoma should be changed to benign necrotising otitis externa. Microangiopathy has a leading role in the aetiology. Surgery should be conducted in most cases.

Clinical Characteristics and Treatment Outcomes for Patients With External Auditory Canal Cholesteatoma.

OBJECTIVE: We aimed to evaluate the clinical features and treatment outcomes for patients with idiopathic and secondary external auditory canal cholesteatoma (EACC), and to validate the treatment strategy from the perspective of hearing as well as etiology and staging. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center and affiliated hospitals. PATIENTS: Fifty-eight patients with idiopathic EACC and 14 patients with secondary EACC. INTERVENTION: Conservative management and surgery. MAIN OUTCOME MEASURE: Air conduction (AC) pure-tone averages (PTAs) and mean air-bone gaps (ABGs). RESULTS: There were no significant differences between hearing values before and after conservative management for idiopathic EACC patients with stages I-III, indicating that hearing abilities were preserved. For idiopathic EACC patients with stage IV disease treated with surgery, the AC PTA threshold and mean ABG significantly improved from a preoperative value of 60.3 dB HL to a postoperative value of 32.4 dB HL (p = 0.013), and from 34.3 to 9.5 dB HL (p < 0.001), respectively. For secondary EACC, the AC PTA threshold and mean ABG significantly improved from a preoperative value of 49.5 dB HL to a postoperative value of 23.2 dB HL (p < 0.001), and from 31.4 to 6.7 dB HL (p < 0.001), respectively. CONCLUSION: The treatment modalities should be selected based on the perspective of hearing as well as the extent of disease and etiology. The early lesions can be treated conservatively, whereas the advanced lesions or cases refractory to conservative management require complete surgical removal of EACC.

Conditions of the external and middle ear: an overview of presentation, management and associated complications.

Ear, nose and throat (ENT) presentations to primary care are common and frequently affect military patients. Many patients can be managed in primary care with appropriate treatment, but some presentations require appropriate, timely, and occasionally emergency onward referral for hospital management. This paper discusses the management of common otological presentations including otitis externa (OE), acute otitis media, chronic suppurative otitis media (including cholesteatoma), tympanic membrane (TM) perforations and pinna haematoma.

Clinical characteristics and follow-up of patients with external ear canal cholesteatoma treated conservatively.

We conducted a retrospective study to evaluate the clinical properties and follow-up of patients with external ear canal cholesteatoma (EECC) who were treated conservatively. Our study group was made up of 15 patients-6 men and 9 women, aged 21 to 82 years (mean: 48). In addition to demographic data, we compiled information on presenting signs and symptoms, the location of the lesion, treatment, and follow-up. All EECCs occurred spontaneously, and all were limited to the temporal bone. Lesions were left-sided in 7 patients, right-sided in 7, and bilateral in 1. For purposes of defining the location of the lesions, the ear canal was divided into four quadrants: anterior, posterior, superior, and inferior. Temporal bone computed tomography (CT) and otomicroscopic evaluation revealed that 7 of the 16 lesions (44%) were located in the anterior and inferior quadrants, 6 (38%) in the inferior quadrant only, 2 (13%) in the anterior, inferior, and posterior quadrants, and 1 (6%) in the anterior quadrant only. Otorrhea was present in 7 of the 15 patients (47%), otalgia in 6 (40%), itching in 4 (27%), fullness in 2 (13%), and occlusion in 1 (7%); 3 patients (20%) were asymptomatic. All patients were treated with local debridement and aspiration under otomicroscopy, and they were followed up with repeat aspirations approximately every 10 weeks under microscopy. The duration of follow-up ranged from 6 to 75 months (mean: 41). At study's end, cholesteatoma had not progressed in any patient during follow-up, indicating that repeat aspirations and regular follow-up of limited EECC prevent recurrence of signs and symptoms and progression of the disease.

Facial extention of ear pathology: infected cholesteatoma causing a parotid abscess.

A man in his early 30s presented with right-sided preauricular swelling and facial oedema. He had a history of acid injury to his right ear as a child resulting in pinna deformity and subsequent blind sac closure of the external auditory canal. Imaging showed abnormal ear anatomy and abnormal density of the right parotid gland. Antibiotic therapy prevented progression but did not resolve the symptoms. Therefore, the infected area was surgically drained. This showed an underlying cholesteatoma, a benign but locally destructive condition where keratinising squamous epithelium grows in the middle ear and mastoid. The infected region was drained and the cholesteatoma was excised. This led to full resolution of the infection. The patient is awaiting a follow-up diffusion-weighted MRI. This case was unusual as the disease had extended beyond the ear and we therefore wish to alert clinicians to cholesteatoma as a possible cause of facial swelling.

Infectious causes of cholesteatoma and treatment of infected ossicles prior to reimplantation by hydrostatic high-pressure inactivation.

Chronic inflammation, which is caused by recurrent infections, is one of the factors contributing to the pathogenesis of cholesteatoma. If reimplantation of autologous ossicles after a surgical intervention is intended, inactivation of planktonic bacteria and biofilms is desirable. High hydrostatic pressure treatment is a procedure, which has been used to inactivate cholesteatoma cells on ossicles. Here we discuss the potential inactivating effect of high hydrostatic pressure on microbial pathogens including biofilms. Recent experimental data suggest an incomplete inactivation at a pressure level, which is tolerable for the bone substance of ossicles and results at least in a considerable reduction of pathogen load. Further studies are necessary to access how far this quantitative reduction of pathogens is sufficient to prevent ongoing chronic infections, for example, due to forming of biofilms.

Some considerations in congenital cholesteatoma.

PURPOSE OF REVIEW: A review of recent publications is combined with our clinical experience to provide salient tips for optimizing the care of children with congenital cholesteatoma. RECENT FINDINGS: Increasing utilization of surgical adjuncts such as endoscopy and KTP laser provides the opportunity to improve postoperative outcomes by reducing residual cholesteatoma rates and improving hearing outcomes. Nevertheless, the best prospect of leaving the child with a normal ear is achieved by prompt removal after early detection when the lesion is still small. SUMMARY: Education of primary care providers to promote early identification and referral of congenital cholesteatoma remains an important objective and responsibility for otolaryngologists in order to obtain optimal outcome.

Cost of cholesteatoma care at a tertiary medical center.

OBJECTIVE: Estimate the available direct cost of cholesteatoma care in a university practice. STUDY DESIGN: Retrospective review of both physician and hospital financial data during a recent 3-year period. SETTING: University-based tertiary referral medical system. PATIENTS: Adults (≥ 18 yr old) with cholesteatoma. INTERVENTION(S): Financial information associated with both physician and hospital encounters were analyzed in a deidentified manner. MAIN OUTCOME MEASURE(S): Frequency and type of encounter, charges, collections, and payers were tabulated. RESULTS: Approximately 949 physician encounters (817 clinic, 130 surgical, and 2 inpatient) among 344 patients resulted in greater than $700, 000 in charges and greater than $211,000 in receipts (≈ 30% rate of collection). The average physician charge per patient per year was approximately $1,600. About 259 hospital encounters among 171 patients resulted in greater than $1.8 million in charges and greater than $520,000 in receipts (≈ 28% collection rate). The average hospital charge per patient per year was ∼$10,000. For physician encounters, managed care (37%) and Medicare (25%) were the most common payers, whereas 17% were uninsured. For hospital encounters, managed care (28%) and Medicare (14%) were the most common payers, whereas 24% were uninsured. CONCLUSION: The direct cost of care for patients with cholesteatoma is significant. The current treatment paradigm for this chronic disorder results in repeated health care system access and associated direct (and unmeasured indirect) expenses. Future treatment paradigms should be designed to improve disease-specific quality of life while mitigating this financial impact.

Absceso cerebral en niños: una complicación de otomastoiditis crónica de diagnóstico tardío / Brain abscess in children: a complication of late diagnosis chronic mastoiditis

Reportamos el caso de una paciente de 7 años de edad que presentó otitis media aguda complicada con otomastoiditis crónica quien fue operada de una timpanomastoidectomía a los dos meses y medio de enfermedad, a los tres meses presentó fiebre, signos de hipertensión endocraneana y paresia del miembro superior izquierdo. La tomografía espiral multicorte mostró absceso en el lóbulo temporal ipsilateral. Recibió tratamiento antibiótico de amplia cobertura y fue sometida a resección quirúrgica del absceso. Seis semanas después de la intervención quirúrgica salió de alta con monoparesia del brazo izquierdo con desempeño independiente.

We report a case of a seven year old patient who had acute media otitis complicated with chronic otomastoiditis, who was operated of timpanomastoidectomy after two months and a half of illness, at three months she had fever, endocraneal hypertension signs and paresia of the left upper limb. The multislice spiral tomography showed the presence of an abscess on the ipsilateral temporal lobe. She received extended-spectrum antibiotic therapy and surgical excision of the abscess. Six weeks after the surgery she was discharged from the hospital with left upper limb monoparesia with independent performance.

Adenoid cystic carcinoma of the external auditory canal associated with cholesteatoma in an 8-year-old girl.

Adenoid cystic carcinoma of the external auditory canal is very rare in a child, and has not previously been reported in an individual younger than 18 years. We report an 8-year-old girl with adenoid cystic carcinoma of the external auditory canal associated with cholesteatoma. At the time of diagnosis, the tumor had invaded the surrounding structures and metastasized to the lymph nodes. She underwent palliative surgery and radiotherapy. She subsequently deteriorated and died of her disease 31 months later.

Management of labyrinthine fistulae in Kyoto University Hospital.

CONCLUSION: In cases of labyrinthine fistulae, we performed complete removal of the cholesteatoma matrix in a one-stage procedure, resulting in a satisfactory bone conduction (BC) hearing preservation rate. Preoperative evaluation of labyrinthine fistulae using high resolution computed tomography (HRCT) detected 86% of cases, and this contributed to favorable results achieved with the surgical treatment of labyrinthine fistulae. We aimed to review cases of labyrinthine fistulae to summarize their outcomes and establish standards of management. METHODS: This was a retrospective chart review of 22 patients with labyrinthine fistulae at Kyoto University Hospital from 2001 to 2009. Patient background (age and sex), location and stage of the fistulae, facial nerve status, preoperative and postoperative BC hearing levels, preoperative CT diagnosis, and surgical procedures were analyzed. RESULTS: The incidence rate of the labyrinthine fistulae was 11.2%. All but one patient had labyrinthine fistula due to cholesteatoma. The fistulae were found in the lateral semicircular canal in 17 cases (77%) and in multiple organs in 4 cases (18%). The BC hearing level was preoperatively scaled out in seven cases. Preoperative HRCT scan revealed the presence of fistulae in 19 cases (86%). For all cases of cholesteatoma, the matrix was completely removed in a one-stage procedure and the fistulae were sealed using bone pate, temporal fascia, and temporal bones. Of the 15 cases with residual BC hearing ability, BC hearing was preserved in up to 12 cases. Two cases with postoperative deterioration of BC hearing had stage 4 fistulae in the cochleae.

Ear canal cholesteatoma: meta-analysis of clinical characteristics with update on classification, staging and treatment.

PURPOSE OF REVIEW: We present an update on clinical evaluation, staging, classification and treatment of canal cholesteatoma, including a meta-analysis of clinical data of the last 30 years. RECENT FINDINGS: Ear canal cholesteatoma is frequently associated secondarily to other canal pathologies. The cause for the rare idiopathic form of the disease remains enigmatic. Epidemiologic and experimental studies of its pathogenesis have increased; however, the main explanatory theory of a deficient migratory capacity of the canal epithelium affected has been falsified only recently. Therefore, the debate on the pathogenesis has gained additional impetus and more data is needed. SUMMARY: Canal cholesteatoma is a rarity in otologic pathology, often leading to misdiagnosis as external otitis or otomycosis by physicians unfamiliar with the disease. It presents typically with otorrhea, focal erosion and keratin accumulation in the osseous ear canal and has to be distinguished from keratosis obturans, which leads to otalgia and bilateral conductive hearing loss by ceruminal plugs, with circumferential distention of the ear canal. Treatment by canaloplasty is curative and highly successful. Alternative conservative treatment is feasible, however, requiring long-term follow up, with often painful cleaning of the lesion.

Therapeutic approaches to complicated cholesteatoma of the external auditory canal: a case of associated facial paresis.

Spontaneous cholesteatoma of the external auditory canal (EAC) is an uncommon condition that is difficult to diagnose. In a patient with such a possibility, serious clinical investigation along with radiologic and histologic exploration should be performed early on because a delay in treatment can lead to severe complications. Given the rarity of EAC cholesteatoma, no therapeutic consensus has emerged. The type of management depends on the extensiveness of invasion and bone erosion and the status of the neighboring structures. The primary therapeutic objectives are to eradicate the cholesteatoma and then to fill in the residual cavity, which in our opinion can be best accomplished with a muscle flap and EAC reconstruction. Postoperative follow-up should be carried out to look for infections, stenosis, and recurrence. We report a new case of spontaneous EAC cholesteatoma, and we review its diagnostic and therapeutic challenges.

Transtemporal approach to otogenic brain abscesses.

The standard current treatment of an otogenic brain abscess is drainage via neurosurgical burr hole or complete excision, followed by an otological procedure to eradicate the primary pathology ­ often at a later date. We describe the drainage of otogenic brain abscess via a transtemporal approach. We present a retrospective study of six cases, five children and one adult. All the children had acute middle ear disease while the adult patient had a petrous apex cholesteatoma. All cases had an otogenic intracranial abscess either in the posterior cranial fossa or in both posterior and middle cranial fossae. Diagnosis was confirmed by computed axial tomography (CT) scan. All the patients were treated by mastoidectomy and needle aspiration to drain the abscesses. In all cases the brain abscess and the ear pathology were successfully treated by a single stage transmastoid approach. The five paediatric patients had an extended cortical mastoidectomy approach to both intracranial pathology and ear disease except one patient who required a burr hole to drain a posteriorly located subdural posterior fossa abscess. The adult patient underwent petrosectomy, followed by transtemporal abscess drainage. There was postoperative pus recollection in one patient who required further aspiration. We conclude that transtemporal drainage of an otogenic brain abscess can successfully treat otological and intracranial pathology in a single operation. It has a low complication rate and avoids the need for a craniotomy or subsequent operations.

[Cholesteatoma of external auditory canal among 18 cases].

OBJECTIVE: To improve the diagnosis and treatment of the external auditory canal cholesteatoma (EACC). METHOD: The data of 18 patients caused by EACC were analysed retrospectively. RESULT: In all cases, the cholesteatomas were found in the external auditory canal and the mastoid cavity. CT scanning was using for definition the ranging of lesions and the timm management surgery was completed. There was no recurrence in 10-40 months follow-up. CONCLUSION: EACC is easily misdiagnosed as keratosis obturans (KO). The disease can progress to extensive mastoid destruction. CT of temporal bone and detailed history is necessary in the diagnosis. Early complete surgical treatment is the best method.