CLINICAL REPORT Indian Journal of Otolaryngology and Head & Neck Surgery https://doi.org/10.1007/s12070-024-04702-y Case Report A 29-year-old male patient presented to our outpatient department (OPD) with complaints of sudden right ear pain and persistent right ear discharge for the past three weeks. The ear pain was described as a sudden, deep, throbbing sensation accompanied by profuse, foul-smelling, mucopu- rulent discharge that was not blood-stained. Approximately one week later, the patient noticed facial asymmetry. Upon examination, the right ear canal appeared edematous and filled with granulation tissue and pus. A grade 5 facial palsy, according to the House-Brachmann classification, was observed. Figure 1a. A high-resolution computed tomography (HRCT) of the temporal bone was performed, revealing opacification in the ear canal, middle ear, and aditus without evidence of cav- ity formation Fig. 2a, b. Notably, the horizontal segment of the facial nerve showed dehiscence Fig. 2c. The initial diag- nosis was acute suppurative otitis media with facial nerve palsy, prompting the patient to undergo mastoid exploration and facial nerve decompression. During the intraoperative examination under a micro- scope, a total perforation with extensive destruction of soft tissue and necrosis of the external auditory canal (EAC) skin was observed Fig. 4. The mastoid antrum was filled with granulation tissue, and the remaining mastoid air cells Introduction Extramedullary manifestations of acute myeloid leukemia (AML) in the temporal bone are rare but significant. While AML primarily affects the bone marrow, there are instances where leukemic cells infiltrate the temporal bone, leading to various complications such as hearing loss, facial nerve dysfunction, balance problems, headaches, and intracranial pressure issues [1]. Diagnosis involves a combination of clinical evaluation, imaging, and biopsy. Treatment, typi- cally overseen by a multidisciplinary team, may encompass chemotherapy, radiation therapy, and surgical interventions [2, 3]. The prognosis varies depending on the extent of the disease and the patient’s response to treatment. The extra- medullary manifestation of Acute Myeloid Leukemia poses a unique diagnostic challenge. In this case report, we pres- ent an intriguing case of a 29-year-old male patient who initially presented with symptoms resembling acute suppu- rative otitis media with facial palsy, only to be later diag- nosed with temporal bone chloroma.  S B Nidhin sbnidhin@gmail.com 1 Department of ENT & HNS, AIIMS Raipur, Raipur, India Abstract Acute Myeloid Leukemia (AML) is the most common leukemia observed in the adult population, accounting for 80% of all leukemia cases. Extramedullary involvement in AML, where leukemic cells are found in organs or tissues outside the blood or bone marrow, is a rare occurrence [1]. The most frequent sites of extramedullary disease include the skin, central nervous system (CNS), and lymph nodes [2, 3]. In this case report, we present an instance of extramedullary AML in the temporal bone, which initially presented with symptoms such as earache, discharge, and facial asymmetry, mimicking acute suppurative otitis media with facial palsy. The patient underwent mastoid exploration and facial nerve decompres- sion. A post-operative bone marrow biopsy confirmed the diagnosis of AML, leading to the initiation of chemotherapy. The patient is currently under follow-up care. Keywords Extra medullary acute myeloid leukemia · Facial palsy · Temporal bone myeloid sarcoma Received: 20 January 2024 / Accepted: 12 April 2024 © Association of Otolaryngologists of India 2024 Atypical Presentation of Acute Suppurative Otitis Media with Facial Palsy: Extra Medullary Manifestation of AML in Temporal Bone Ripu Daman Arora 1  · S B Nidhin 1  · Nitin M Nagarkar 1  · Amit Kumar Banjare 1 1 3