A case of bilateral eosinophilic granuloma in the temporal bone.

We present a case of bilateral eosinophilic granuloma in the temporal bone in a 47-year-old woman, who visited our hospital with a headache and a feeling of occlusion in her left ear. Her left tympanic membrane was slightly turbid and pure tone audiometry revealed mild left sensorineural deafness. CT disclosed a shadow of soft tissue in the left mastoid antrum and mastoid cells, which was indicative of marked destruction of the bone. Because MRI findings led us to suspect otitis media cholesteatoma, a mastoidectomy was performed. The mastoid antrum and mastoid cells were filled with easily bleeding granulation, and there was a wide range of bone deficit in the posterior cranial fossa. Histopathologically, the granulation tissue was an eosinophilic granuloma. Her postoperative clinical progress was good and she was discharged. However 2 months after discharge, she had a feeling of occlusion in the right ear and CT revealed a shadow in the right mastoid antrum and cells. Therefore, right tympanoplasty was performed and the same findings as in the left ear were obtained. A histopathological diagnosis of eosinophilic granuloma was made again. To date, there has been no recurrence.     

Temporal bone pathology of adult-type osteopetrosis

We present the pathologic features in the temporal bones of a 62-year-old woman with the adult benign form of osteopetrosis. Most of the bony tissue was expanded by dense lamellar bone, with, in some places, the presence of residual calcified cartilage. In the otic capsule, globuli interossei were greatly increased in number. The ossicles were enlarged with fixation of the stapes. Narrowing of mastoid air cells, the internal auditory meati, and eustachian tubes was present, the latter associated with chronic otitis media. The bone deposition in the ossicles contributed to the conductive hearing loss, which was a prominent feature in this patient's otologic findings. The narrowing of the internal auditory meati may similarly have contributed to a degree of sensorineural hearing loss.     

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.     

仅存单耳听力的中耳手术

目的探讨仅存单耳听力的中耳手术和听力重建的可行性。方法回顾分析2005-2011年间手术的12例仅存单耳听力的中耳手术后的听力改善情况。其中9例完成开放式鼓室成形术,2例行完桥式鼓室成形术,1例完成外耳道切除＋游离皮瓣修复＋鼓室成形术。结果所有病例随访不少于1年,12例均干耳。语频段听力测试气骨导差缩小30dB以上者3例,20dB以上者4例,10dB以上者3例,2例听力与术前基本相同,未发生术耳感音神经性聋并发症。结论对于仅存单耳听力的中耳手术是可行的,同期可以完成听力重建维持或提高现有听力,术中尽量避免对内耳可能的损伤,减少感音神经性聋的并发症。     

Otologic disease in AIDS patients: CT correlation

The contrast-enhanced computed tomographic (CT) scans of the temporal bone and brain in 18 patients with otologic disease and acquired immunodeficiency syndrome (AIDS) were retrospectively reviewed. Seven scans revealed middle ear and mastoid disease; three scans were consistent with central nervous system (CNS) pathology; and eight scans demonstrated no abnormalities. CT scanning was found useful in localizing otopathology and diagnosing CNS toxoplasmosis, aural polyps, osteomyelitis, mastoiditis, and middle ear effusion due to hypertrophic lymphoid tissue. The authors conclude that AIDS patients with sensorineural hearing loss should undergo contrast-enhanced brain CT scans to rule out CNS pathology; AIDS status does not alter criteria for CT scanning in patients with conductive hearing loss; and that images of the nasopharynx should be included on temporal bone CT scans of patients with conductive hearing loss in order to exclude eustachian tube obstruction by hypertrophic lymphoid tissue.     

Langerhans' cell histiocytosis--a rare cause of sudden onset unilateral sensorineural hearing loss

Langerhans' cell histiocytosis is a rare disorder of unknown aetiology in which pathological Langerhans' cells accumulate and destroy local tissue. We report a 38-year-old female who presented with a sudden onset of left sensorineural hearing loss. Magnetic resonance imaging (MRI) revealed a contrast-enhancing lesion in the left mastoid and a second lesion in the hypothalamus. Following left mastoid exploration and biopsy a definitive diagnosis of Langerhans' cell histiocytosis was made and the patient was treated with external beam radiotherapy. Subsequent right femur and right mastoid involvement were successfully treated with steroids and cytotoxic chemotherapy. At one year follow-up the patient had residual left-sided sensorineural hearing loss with normal hearing in the right ear. To our knowledge, Langerhans' cell histiocytosis has not been previously reported as a cause of unilateral sudden onset sensorineural hearing loss. It should be considered in the differential diagnosis of this condition.     

CT对慢性化脓性中耳炎的诊断意义

目的探讨中耳和乳突不同病变组织术前诊断和鉴别诊断方法。方法回顾分析106例慢性化脓性中耳炎（含胆脂瘤中耳炎）患者颞骨薄层CT显示的病变组织，并进行183次CT值测量，与术中发现、术后病理进行对比分析，得出不同病变组织的CT值范围。从中随机抽取60例，由同一组医师根据不同病变组织CT值及周围组织破坏的情况，重新分析做出诊断，将其诊断符合率与放射科常规诊断报告进行比较。根据临床表现和颞骨薄层CT扫描结果对9例慢性化脓性中耳炎患者行预测性诊断，以验证本研究结果对于中耳、乳突不同病变组织术前诊断与鉴别诊断的价值。结果胆脂瘤、肉芽组织、胆脂瘤并肉芽组织、炎性渗出、硬化或钙化组织、黏膜增厚并息肉样变组织的CT值分别为：（46．6±10、3）Hu、（26．6±7．4）Hu、（42．1±11．4）Hu、（-24．6±9．2）Hu、（223．6±63．7）Hu、（23．8±8．5）Hu。根据不同病变组织CT值大小及周围组织的破坏情况对60例患者重新分析诊断，诊断符合率从68．3％提高至81．7％（Х^2=3．96，P〈0．05）。根据临床表现和颞骨薄层CT扫描所见进行预测性诊断，诊断符合率达90．0％。结论CT值虽不能作为诊断和鉴别诊断中耳、乳突不同病变组织惟一可靠的依据，但不失为一种很有意义的线索；根据临床表现、颞骨薄层CT扫描所见对中耳各种病变进行综合分析，可显著提高中耳、乳突病变诊断和鉴别诊断的准确率。     

279例耳闷胀感的病因分析

目的 探讨产生耳闷胀感的部位和原因。方法 收集耳闷胀感为主诉的279例362耳,对患耳的临床表现、鼓室导抗图、纯音测听和咽鼓管功能进行分析。结果 362耳中鼓室导抗图A型203耳、B型111耳、C型33耳以及鼓膜穿孔15耳。纯音测听表现为传导性聋、感音神经性聋、混合性聋以及正常者分别是83耳、8l耳、105耳和93耳。在362耳中,咽鼓管功能异常208耳,在A型鼓室导抗图203耳中,咽鼓管功能异常67耳。结论 产生耳闷胀感的病变部位包括外、中、内耳和面神经的病变,其中以分泌性中耳炎、各种原因引起的感音神经性聋和“咽鼓管狭窄症”为多。     

Tuberculous otitis media –series of 10 cases

BackgroundTuberculosis (TB) of the middle ear cleft (MEC) is a rare extra-pulmonary manifestation. Signs and symptoms of tuberculous otitis media are indistinguishable from that of non-tuberculous otitis media making early diagnosis difficult.ObjectivesTo study the clinical presentations, complications and effective diagnostic modalities in tuberculosis of middle ear cleft.MethodsWe retrospectively studied 10 patients diagnosed with chronic otitis media, unresponsive to 2 months conventional treatment. Pure tone audiogram, High resolution computed tomography (HRCT) of temporal bone, and AFB staining of ear discharge were done. All patients underwent mastoid surgery. AFB staining and histopathological examination of granulation tissue removed from the middle ear and mastoid were also done.ResultsClinical findings were mastoid swelling, facial palsy and post-aural fistula 3,4 & 2 patients respectively. All patients had persistent ear discharge and three had vertigo. Hearing loss was of moderate conductive type in five, sensorineural type in three and mixed type in two. HRCT of temporal bone revealed soft tissue density in MEC in 9 and evidence of bone destruction in 6 cases. Diagnosis of TB was confirmed either by (a) demonstration of AFB in ear discharge (4 patients)/tissue removed during surgery (4 patients) or (b) by demonstration of tuberculous granulomas with necrosis on histopathological examination of tissue from MEC (8 patients).ConclusionTuberculosis should be suspected in all cases of chronic otitis media unresponsive to conventional treatment particularly in endemic areas. Histopathological examination and AFB staining of tissue removed during mastoid surgery are reliable diagnostic methods.     

中耳胆固醇肉芽肿8例报告

目的 :探讨中耳胆固醇肉芽肿 (CG)的病因、发病机制、诊断及治疗。方法 :回顾性分析 8例 (9耳 )CG患者的临床资料。 8例 (9耳 )均为轻～中度传导性聋 ,鼓膜呈“蓝鼓膜”征 ,鼓室压曲线图为B型 (除 1例鼓膜紧张部穿孔外 ) ,其中 7例 (8耳 )行鼓室探查术 ,开放上鼓室、鼓窦及乳突气房 ,彻底清除肉芽组织 ,有 5例行中耳置管术 ,2例行中耳乳突改良根治术 ;另 1例仅行鼓膜穿刺抽液。结果 :1例失访。 7例 (8耳 )术后随访 0 .5～ 6年 ,6例 (7耳 )无复发 ;1例 (1耳 )复发 ,再次行手术治疗 ,术后 1年无复发。结论 :对不明原因的血性耳溢液及蓝鼓膜 ,应结合CT提高术前诊断率。对CG应采取手术治疗 ,清除病变 ,建立鼓室及乳突的通气、引流。     

隐匿性中耳炎的诊断与治疗

目的 探讨隐匿性中耳炎的诊断及治疗方法,提高隐匿性中耳炎的诊治效果.方法 回顾性分析我院1994年1月～2005年12月经手术治疗和病理证实的15例隐匿性中耳炎患者的临床资料,总结其病因及临床特点.结果 所有病例无明显耳漏病史,鼓膜和外耳道局部无明显阳性体征发现,3例急性发作时有鼓膜充血,1例伴鼓膜穿孔及脓液搏动.听力损失多为传导性聋.颞骨CT扫描示病变主要集中在上鼓室和鼓窦,4例病变充满乳突气房及鼓室、鼓窦.主要症状为听力渐进性下降、头闷痛、耳闷堵塞感及耳鸣等.术中探查中耳气房内有肉芽组织10例,其中4例伴有积液及胆固醇肉芽肿;另2例为胆脂瘤病变.手术主要是清除病变,解除引流通道的阻塞并一期行鼓室成形术.术后随访6个月～5年,病变无复发,听力平均提高16.3 dB.结论 应提高对隐匿性中耳炎的认识,对不明原因的听力下降及局部胀痛不适者应行纯音听力测试、声导抗检查及高分辨率颞骨CT扫描.高分辨率颞骨CT扫描可作为诊断隐匿性中耳炎的主要手段,手术是有效的治疗方法.     

Paget's disease and the temporal bone--a clinical and histopathological review of six temporal bones

Paget's disease of bone occurs more commonly in the elderly and has been reported to involve the temporal bone in 30% of those afflicted. The clinical and histopathological features of six temporal bones from three patients with this disease are reported and the relevant literature reviewed. The effect of Paget's disease on the middle ear structures was more variable than its extension into the otic capsule. Pagetic involvement of the otic capsule was observed in five temporal bones. One patient had bilateral asymptomatic neurofibromas in the eighth cranial nerve. the potential mechanisms responsible for the conductive deafness, the sensorineural deafness and vestibular dysfunction associated with Paget's disease are discussed.     

Advances in auditory implants

Auditory implants are classified into bone conduction (BAHA and Bonebridge; BB) and active middle ear implants (Vibrant Soundbridge; VSB) that stimulate cochlear hair cells, and cochlear implants (CIs) that stimulate neural structures. CIs should be performed as early as possible, and bilateral CIs have become popular because sound localization and speech recognition can be improved. CI is also considered a desirable treatment option for patients with single-sided deafness. VSB provides a safe and effective option for patients with conductive or mixed hearing loss and moderate to severe sensorineural hearing loss (SNHL); however, it use in patients with conductive or mixed hearing loss have only been approved in Japan. BAHA and BB implants have been approved by national insurance in Japan as bone conduction implants for patients with conductive or mixed hearing loss. Two fully implantable devices (Cochlear Carina and Envoy Esteem) are provided for patients with SNHL.     

Tissue engineering for the regeneration of the mastoid air cells: a preliminary in vitro study

Mastoid is a pneumatic bone, composed of small interconnecting chambers covered by a mono-layer of mucosa with an abundant blood supply. One of its main functions is gas exchange according to the concentration/pressure gradient. The final goal of our research project is to regenerate mastoid air cells and their unique physiologic functions. The aim of the present study is to determine appropriate cultivating conditions for the cells cultured on the surface of artificial hydroxyapatite. In our in vitro experiment, to imitate the skeleton of mastoid bone, we used two types of three-dimensional hydroxyapatite (3D-HA), i.e. with a high (90%) and low (60%) percentage of micropores. The former type was divided into two groups: collagen-coated and non-coated. Canine mucosal- and bone marrow-derived stromal cells (BSCs), from the oral floor and femur respectively, were harvested and cultured on the 3D-HA under different conditions. To estimate the proliferation/distribution of the cultured cells over the surface of the 3D-HA, these cells were stained with the dye DiI and hematoxylin-eosin. There were no significant differences in the proliferation of cultured cells on the 3D-HA with high and low percentages of micropores. Collagen-coated HA was a better material for the cultured cells compared with the non-coated HA. Co-cultured mucosal and BSCs proliferated better than those cultured separately. In conclusion, this tissue engineering technique may be applied for the regeneration of mastoid air cells.     

Radiation injury to the temporal bone

Osteoradionecrosis of the temporal bone is an unusual sequela of radiation therapy to the head and neck. Symptoms occur many years after the radiation is administered, and progression of the disease is insidious. Hearing loss (sensorineural, conductive, or mixed), otalgia, otorrhea, and even gross tissue extrusion herald this condition. Later, intracranial complications such as meningitis, temporal lobe or cerebellar abscess, and cranial neuropathies may occur. Reported here are five cases of this rare malady representing varying degrees of the disease process. They include a case of radiation-induced necrosis of the tympanic ring with persistent squamous debris in the external auditory canal and middle ear. Another case demonstrates the progression of radiation otitis media to mastoiditis with bony sequestration. Further progression of the disease process is seen in a third case that evolved into multiple cranial neuropathies from skull base destruction. Treatment includes systemic antibiotics, local wound care, and debridement in cases of localized tissue involvement. More extensive debridement with removal of sequestrations, abscess drainage, reconstruction with vascularized tissue from regional flaps, and mastoid obliteration may be warranted for severe cases. Hyperbaric oxygen therapy has provided limited benefit.     

结核性中耳乳突炎的临床特征及疗效分析

目的 探讨结核性中耳乳突炎的临床特点以及手术疗效.方法 回顾性分析经病理学证实的16例(18耳)结核性中耳乳突炎的临床表现、影像学特征以及治疗方法,并随访其预后.结果 16例(18耳)患者均表现为耳流脓、听力下降,并发重度感音神经性聋患者3例(4耳),并发周围性面神经麻痹患者3例.16例颞骨高分辨率CT均表现为鼓室、乳突充满软组织密度影,其中骨质破坏11例(12耳),颞骨内存在死骨7例(7耳).16例患者中伴发肺结核7例.15例患者接受了手术治疗去除病灶并联合抗结核治疗.除2例患者术前经穿孔的鼓膜取活检明确诊断为结核性中耳乳突炎外,其余14例患者均为术中或者术后确诊.除1例患者失访外其余15例患者均随访1年以上,中耳乳突结核无复发,3例面神经麻痹患者基本恢复正常.结论 对于顽固性耳流脓,影像学提示鼓室、乳突充满软组织密度影且存在骨质破坏或者死骨者,应仔细询问是否有结核病史,以便鉴别结核性中耳乳突炎.手术去除病灶并结合抗结核治疗对该病有较高的治愈率.     

Clinical aspects of the osteolytic (inflammatory) phase of cochlear otosclerosis

During the last years it has been demonstrated that the active stage of otosclerosis (French: otospongieuse) is caused by an osteolytic inflammation associated with a measles-virus infection. Under influences that are not yet well understood (e.g. estrogens), the osteolytic process is arrested and changed to osteoblast activity accompanied by new bone formation. This latter process can be understood as a scar formation. If the process takes place within the area of the oval window, it results in fixation of the stapes and conductive hearing loss. When the otosclerotic process is restricted to the cochlea, clinical signs are not well defined. We report on exemplary cases of progressive bilateral sensorineural hearing loss caused by otosclerosis, restricted to the cochlea. The characteristics of the clinical course include: 1. Bilateral asymmetric sensorineural hearing loss. 2. Roaring tinnitus. 3. Episodes of sudden hearing loss. 4. Excellent response to prednisolone therapy. 5. In the late stage of the disease a small but transitory conductive hearing loss. High resolution computed tomography in such cases reveals diffuse osteolytic foci within the cochlear walls. In severe cases of such progressive deafness we discuss an antiproliferative (immunosuppressive) therapy with cyclosporine and/or a radionuclide therapy with Sn-117 m, an isotope with a very small irradiation radius.     

Atresia and sudden sensorineural hearing loss

This case study summarizes findings in an adult male, aged 57, who presented to the Adult Audiology Clinic with aural atresia in the right ear resulting in a conductive hearing loss and a sudden sensorineural hearing loss in the left ear. Treatment options included reconstruction surgery in the right ear, bone anchored hearing aid in the right ear to overcome the conductive hearing loss, bone anchored hearing aid in the left ear for single sided deafness, and intratympanic steroid injections in the left ear to salvage hearing. This case study highlights that when a patient is educated on all available options the patient is then able to make a decision comfortable to him and to help improve his hearing.     

Masking Level Difference in Normal and Pathological Ears

The masking level difference (MLD) was studied in 9 normal subjects, in 15 patients with conductive hearing losses, in 9 subjects suffering from Menière's disease, and in 8 subjects with sensorineural hearing losses

Results showed that MLD's size not depend on the conductive impairement but it may be affected by the degree of the deafness both in Menière's disease and in sensorineural lesions     

Wildervanck's syndrome--unilateral Mondini dysplasia identified by computed tomography

We present a case of the Wildervanck (cervico-oculo-acoustic) syndrome exhibiting congenital deafness. Klippel-Feil anomaly and lateral rectus palsy with enophthalmos. Audiometry indicated a predominantly conductive loss which, because of masking difficulties, was assumed to be bilateral: an erroneous assumption supported by results of conventional petrous bone tomography which failed to demonstrate any abnormality of the inner ears. Computed tomography (CT), however, revealed a severe Mondini dysplasia of one ear, a condition which must be assumed to be associated with severe sensorineural hearing loss. Reconstructive middle ear surgery for the conductive loss on the other side was therefore contraindicated.